Special birth defects and neurological diseases can cause neurogenic detrusor overactivity (NDO). First-line pharmacotherapy is the antimuscarinic therapy, which can be limited by side effects or non-effectiveness. Therefore, pharmacological treatment of NDO in children includes off-label use of intravesical injections of botulinum toxin type A (BTA). In this review article, various clinical studies in which BTA was used for the treatment of NDO of different etiologies in children are discussed, including studies about children with acquired NDO. An electronic literature search was performed using PubMed, and studies published prior to March 2019 are reported. BTA injections are a clinically and urodynamically effective and safe treatment for NDO in children. The treatment is also important in protecting the upper urinary tract from damage and improving concomitant bowel dysfunctions. Fibrotic, acontractile bladders with poor bladder compliance and/or a very small initial bladder capacity and/or the presence of an open bladder neck all contributed to poor responses. A combined injection into the detrusor and external urethral sphincter may improve the clinical outcome in the detrusor–sphincter dyssynergia. New application methods are promising, such as the electromotive drug administration, to avoid general anesthesia. Furthermore, the minimal clinically effec-tive dosage, inclusion criteria, and prognostic factors remain to be established.