Objectives/BackgroundHigh‐output stoma is one of the most common major morbidities in young children with an enterostomy that could lead to intestinal failure. Management of high‐output enterostomy in children is mostly based on personal experience. This systematic review aims to clarify the evidence‐based therapeutic approach of high‐output enterostomy in children.MethodsA systematic review was performed using Pubmed, Embase (Ovid), and Cochrane Library to identify studies published until March 20, 2023, following the 2020 Preferred Reporting Items for Systematic Reviews and Meta‐Analyses guideline. The study population comprised children (i.e., age <18 years) with high‐output enterostomy (i.e., jejuno‐, ileo‐, and/or colostomy), regardless of underlying aetiology. Interventions comprised any (non)pharmacological and/or surgical treatment. Interventions were compared with each other, placebos, and/or no interventions. Primary outcome was reduction of enterostomy output. Secondary outcomes were morbidity, mortality, quality of life, associated healthcare costs, and adverse events.ResultsThe literature search identified 4278 original articles of which 366 were screened on full text, revealing that none of the articles met the inclusion criteria.ConclusionThis first systematic review on management of high‐output enterostomy in children revealed that any evidence on the primary and secondary outcomes is lacking. There is an urgent need for evidence on conservative treatment strategies including fluid restrictions, dietary advices, oral rehydration solution, chyme re‐infusion, and pharmacological and surgical treatments of high‐output enterostomy in children, aiming to reduce the risk for short‐ and long‐term complications. Till more evidence is available, a systematic and multidisciplinary step‐up approach is needed. Therefore, a therapeutic work‐up is proposed that could guide the care.