Mucormycosis is an increasingly emerging life-threatening infection and is an important cause of morbidity and mortality in patients with leukemia. We present the case of a 4-year-old boy with an acute lymphoblastic leukemia complicated by severe and prolonged neutropenia during induction chemotherapy, who developed cutaneous mucormycosis of the elbow. Direct microscopy with KOH and the histopathologic observation of the skin revealed fungal hyphae without septations. The cultures were positive for Rhizopus microsporus var oligosporus. The patient was treated succefully with amphotericin B during 40 days, surgical debridement and dermoepidermic graft. Early recognition and prompt intervention with combined medical and surgical treatment may improve the outcome. The most common management strategy in survivors involves a combination of antifungal therapy with amphotericin B and surgical debridement.