Key Clinical MessageTransverse colonic volvulus (TCV) is a serious condition with a mortality rate of up to 33%. It is very rare, especially in children. Despite its rarity, surgeons should have a high index of suspicion and include it in the list of differential diagnoses, especially in patients with developmental delays and associated uncommon syndromes. Resection and anastomosis, whether as a one‐stage or two‐stage procedure, proved to be the best treatment options for children. Since prompt identification and management are vital, this paper presents useful information on the presentation, treatment, and outcome of this case report.