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Phaeohyphomycosis refers to infections caused by phaeoid fungi that can have an aggressive course in normal hosts. We report a case of left-sided renal phaeohyphomycosis due to Bipolaris spicifera in a 7-year-old immunocompetent male child. He presented with fever, dysuria, nausea, vomiting and flank pain. Examination revealed tenderness at the left costovertebral angle. Histological examination and culture of biopsy from left kidney and blood yielded the fungal pathogen Bipolaris spicifera. His past history revealed that he was diagnosed perinatally with bilateral hydronephrosis due to bilateral pelvic ureteric junction obstruction. He underwent an open dismembered pyeloplasty on the left side followed by the right side pyeloplasty at the age of 6 months and 1.5 years respectively. He was on a regular follow-up for 5 years and had been doing well. Now he was diagnosed as a case of unilateral renal phaeohyphomycosis. The patient was managed successfully with antifungal drugs amphotericin B and itraconazole. A review of previously reported bipolaris cases with their clinical manifestations, treatment and outcome is presented. Renal phaeohyphomycosis remains an unusual disease. Aggressive diagnostic approaches and careful management helped in survival of the patient.