Primary renal mucormycosis is a rare infection capable of acute illness with sepsis. Few cases have been reported. We report a case of an acute primary renal mucormycosis and review the published reports. The incidence of primary renal mucormycosis has risen in recent years. The most frequently reported underlying predisposing disorders are human immunodeficiency virus infection, intravenous drug abuse, and diabetes mellitus. Primary renal mucormycosis should be suspected in patients with an immunocompromising illness or particular risk factors, when persistent flank pain and fever with sterile urine not responding to appropriate antibiotics are associated with enlarged heterogeneous kidneys.