Renal mucormyosis is a rare entity and normally diagnosed at postmortem. Isolated cases diagnosed antemortem are even rarer and only a few cases have been reported. The disease is associated with a high mortality rate, especially in patients with underlying disease. Clinical presentation is non-specific and includes flank pain, fever and pyuria with or without haematuria. Identifying fungal hyphae microscopically is essential for diagnosis. We present a rare isolated fatal case of renal mucormycosis in a 77-year-old man with chronic obstructive pulmonary disease (COPD) and old tuberculosis (TB) who had been diagnosed after a right nephrectomy. We believe early diagnosis and appropriate treatment for fungal infection, together with the predisposing underlying disease, is crucial for survival.