Basidiobolomycosis is a rare disease due to fungus Basidiobolus ranarum, an environmental saprophyte that is found worldwide, though mainly reported in the tropical and subtropical regions. Basidiobolomycosis is an unusual fungal skin infection, rarely involves the gastrointestinal (GI) tract. Most of the cases of paediatric GI basidiobolomycosis (GIB) were reported from the southern region of Saudi Arabia. We report an 11-year-old Saudi boy. He presented with a huge right lower quadrant abdominal mass and marked eosinophilia. Abdominal computed tomography scan revealed a large caecal mass. A biopsy was taken and it showed transmural granulomatous inflammation. A diagnosis of GIB was confirmed by specific features in histopathology. Most of the reported paediatric cases with GIB required adjuvant therapy of antifungal and surgical resection. In our case, treatment with voriconazole alone for 1 year was successful with complete recovery and with no recurrence after a year of discontinuing the treatment.
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