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Objectives Standard urotherapy (SU) is regularly used as a first-line intervention for children with incontinence. It is suggested that SU is also effective for children with neurodevelopment disabilities (NDs) when altered to the specific needs of these children. The aim of the review was to answer the following research questions: (a) what was the effectiveness of SU interventions in children with ND?, (b) which procedures and protocols were utilized during SU?, (c) what was the role of parents during SU?, and (d) what was the current evidence base regarding SU and children with NDs? Methods Four electronic databases (i.e., Embase, PsychInfo, PubMed, Web of Science) were searched in accordance with the JBI methodology for mixed methods systematic reviews (MMRS) in the Joanna Briggs Institute (JBI) manual for Evidence Synthesis. The Mixed Method Appraisal Tool (MMAT) from JBI was utilized to determine the methodological quality of the studies. Results The search resulted in the inclusion of five articles related to SU in children with ND, indicating that research relating to SU and NDs is scarce. Results of the review indicate that SU resulted in a complete or partial positive response in 30% of the children with NDs (N = 114) based on the criteria listed by the International Children’s Continence Society (ICCS). When using criteria by the authors of the included articles, the results indicate that 59% of the children achieved full continence. Most importantly, the evidence suggests that most children showed improved symptoms (e.g., reduction in urinary/fecal accidents, schedule-dependent continence) even if full continence was not obtained. Conclusions The review indicated that SU could be beneficial as a first-line treatment for children with NDs. Individualizing treatment to the needs of the child, parental involvement, follow-up appointments, and adding adjuncts when SU alone is not effective seem to be beneficial for this specific population. However, research relating to this topic is scarce and therefore more research needs to be completed regarding the role of parents and effective guidelines specific to different NDs. Implications for future research are discussed.