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Ileosigmoidal knotting (ISK) is a rare, possibly fatal cause of intestinal obstruction. ISK is a compound volvulus that is more common in Africa and Asia. ISK is mostly seen in adults, pediatric cases reported in the literature are much rarer. In this report, we present the first reported case of ISK in a pediatric patient from Nepal. An 8-year-old male child presented with symptoms of abdominal pain, vomiting, and obstipation. The abdomen was distended with generalized tenderness. Erect abdominal X-ray showed multiple air-fluid levels. Intraoperatively, gangrenous ileum loops were entangled around the sigmoid, and resection of the gangrenous ileum and sigmoid was performed. An end-to-end colo-colic anastomosis from the descending colon to the remaining sigmoid with a double-loop ileostomy was performed. Pediatric ISK is a rare fatal form of intestinal obstruction that progresses quickly to gangrene. Clinical signs and symptoms are nonspecific, making preoperative diagnosis challenging. [ABSTRACT FROM AUTHOR]