Introduction Neurogenic bladder sphincter dysfunction (NBSD) can develop as a result of a lesion at any level in the nervous system, including the cerebral cortex, spinal cord, or peripheral nervous system. Neurologic conditions in children leading to neurogenic bladder dysfunction are predominantly congenital neural tube defects (including myelomeningocele, lipomeningocele, sacral agenesis, and occult lesions causing tethered cord). Acquired causes such as spinal cord tumors or trauma or sequelae of transverse myelitis are less frequent. Whereas from an etiologic standpoint neurogenic bladder dysfunction is a heterogeneous group, medical management will be similar irrespective of the underlying cause. The vast majority of knowledge about NBSD management comes from long-term experience with myelomeningocele (MMC), the most common neural tube defect. Following the institution of a general treatment policy with advances in neurosurgical and orthopedic treatments in previous decades, governing the associated NBSD has become crucial for improving quality of life and life expectancy in children with neural tube defects. In MMC patients, disordered innervation of the detrusor musculature and external sphincter adversely affects bladder function, which if untreated not only leads to incontinence but also will cause secondary damage and dysfunction of both the upper and lower urinary tracts. Key elements in optimal NBSD management are early diagnosis (including NBSD typology) and early (presymptomatic) institution of adequate medical treatment. There is indeed growing evidence that management decisions made during infancy, which prevent both renal damage and secondary bladder-wall changes, potentially impact long-term outcomes for renal function and safe urinary continence. After describing the pathophysiology of NBSD and its possible consequences, this paper focuses on diagnosis (including early identification of patients at risk), treatment goals, treatment tools, and practical management of NBSD. Historical evolution 1 2 4 5 2 3 6 Pathophysiology of the neurogenic bladder 2 2 2 7 2 2 8 10 2 2 2 11 3 12 13 Management of the neurogenic bladder General principles and treatment goals 14 11 15 16 17 18 Early management, including diagnosis and identification of the high-risk bladder 5 19 20 2 2 3 6 21 22 23 Although many questions regarding optimal evaluation and management remain unanswered, the consensus on the need of close surveillance, especially in the first years of life, plus the possibility that proactive treatment may be better for the bladder in the very long term, emphasize the need for an integrated approach in which clinical observations, serial imaging, and urodynamics are the basis for early adequate treatment. Urodynamic studies: special considerations in children with NBSD 6 24 11 25 11 26 Clean intermittent catheterisation 27 28 29 30 31 32 Pharmacologic treatment: anticholinergics 33 34 35 37 38 43 40 44 41 45 45 46 47 43 48 49 50 51 Medical management of NBSD in clinical practice 52 35 6 29 53 1 Fig. 1 a–d  Once appropriate therapy has been initiated, adequate follow-up is required, with adjustments if needed (CIC frequency, medication dosing and administration route). Treatment efficacy can be assessed using clinical parameters (including CIC frequency and volume charts), urinalysis, renal and bladder ultrasound, X-ray cystography, and video urodynamics. 54 2 55 2 Fig. 2 A a b B C 40  Long-term outcome evaluation and need for life-long follow-up 29 56 56 Conclusions Medical management with CIC and anticholinergics is effective in preserving renal function and providing safe urinary continence in more than 90% of patients with a neurogenic bladder. Early diagnosis and treatment institution, long before continence becomes an issue at toddler age, can prevent both renal damage and secondary bladder-wall changes, thereby improving long-term outcomes. Compared with oral oxybutynin, intravesical oxybutynin has more potent and longer-acting detrusor suppressive effects with good tolerance and should be used prior to considering surgical therapies. Therapeutic goals should no longer be restricted to prevention of secondary damage to both upper and lower urinary tracts. Instead, our goal should be to achieve normal renal and bladder growth at safe bladder pressure, with appliance-free continence.