Bladder management in spina bifida patients relies on clean intermittent catheterization and oral antimuscarinics with a significant failure rate. The efficacy of intradetrusor injections of botulinum toxin has been confirmed in patients with spinal cord injury or multiple sclerosis but not in patients with myelomeningocele. To conduct a systematic review of current evidence regarding the efficacy of intra-detrusor injections of Botulinum Toxin A (BTX-A) in spina bifida patients with neurogenic detrusor overactivity (NDO) refractory to antimuscarinics. A research has been conducted on Medline and Embase using the keywords: ("spina bifida" OR "myelomeningocele" OR "dysraphism") AND "toxin." The search strategy and studies selection were performed using the PICOS method according to the PRISMA statement. Twelve published series were included (n = 293 patients). All patients were <18 years old. There was no randomized study comparing BTX-A versus placebo and most studies had no control group. Most studies reported a clinical and urodynamic improvement with resolution of incontinence in 32-100% of patients, a decrease in maximum detrusor pressure from 32 to 54%, an increase of maximum cystometric capacity from 27 to 162%, and an improvement in bladder compliance of 28-176%. Two studies suggested lower efficacy in patients with low compliance bladder compared to those with isolated detrusor overactivity. Intradetrusor injections of BTX-A could be effective in children with spina bifida but this assumption is not supported by high level of evidence studies. There is no data available in adult patients. Neurourol. Urodynam. 36:557-564, 2017. © 2016 Wiley Periodicals, Inc.