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Gastrointestinal basidiobolomycosis (GIB) is an emerging fungal infection in children that leads to diagnostic confusion.Our study aim was twofold: a systematic review of published literature and an update of some Saudi Arabia hospital series to analyze their as well as our own experience in diagnosis and management of GIB.This clinical study included 18 children whose final diagnosis was GIB. The patients, who ranged in age from 5 to 10 years, were admitted between November 2009 and November 2015 with vague abdominal pains with or without abdominal masses for further investigation.Abdominal pain was the most common presenting symptom (94.4%) followed by constipation and abdominal mass (83.3 and 77.8%, respectively); fever was present in only 22.2% of the cases. Elevated inflammatory markers and eosinophilia (94.4%) appeared as prominent laboratory findings.We conclude that diagnosing GIB in children requires a high index of suspicion, awareness, and consideration of its possibility in the differential diagnosis in patients with abdominal masses and eosinophilia, particularly in areas where it is endemic. Increased awareness of this clinical entity, early surgical resection of the infected tissue, and prolonged treatment with itraconazole offer the best chance for curing the disease.