Herein is described the first case of an infant with neuroblastoma who developed isolated splenorenopancreatic mucormycosis. An 18-month-old boy with neuroblastoma who was on intensive chemotherapy was admitted with febrile neutropenia. On abdominal computed tomography, multiple hypodense lesions in the spleen with invasion to the upper pole of the left kidney were demonstrated. Enlargement of splenic lesions with a complete hypoechoic pattern replacing the whole spleen, consistent with splenic abscess were observed on serial ultrasound. On splenectomy the resected spleen appeared to be severely fragmented and necrotic. On pathology, massive infiltration of broad, non-septate hyphal fragments identified as Mucor with invasion to all blood vessels was seen. Histologically, a piece of the tail of the pancreas also showed involvement by the filamentous Mucor. The present case highlights the necessity of high index of suspicion in susceptible patients, early diagnosis and appropriate management in order to minimize the mortality rate.