Systemic fungal infections with Fusarium occur predominantly in immunocompromised patients and are usually fatal. We report a patient with acute lymphocytic leukemia and fusariosis involving the skin and lungs. This patient underwent antifungal chemotherapy and bilateral pulmonary resections. She subsequently had successful bone marrow transplantation. The results of this treatment suggest that this aggressive management of pulmonary fusariosis offers the best chance of survival.