Isolated renal mucormycosis (IRM) is a life-threatening and extremely rare entity in immunocompetent children. Unfortunately, the diagnosis of mucormycosis using clinical and laboratory parameters can be difficult leading to high mortality. We report a case of IRM in a 3-year-old immunocompetent child who was successfully managed with amphotericin B and nephrectomy. A high index of suspicion, early diagnosis, and aggressive treatment with antifungal and surgery is critical for a favorable outcome.
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