Disseminated fusariosis is an uncommon clinical condition in immunocompromised patients. We report a fatal case of disseminated fusariosis secondary to neuroblastoma in a male patient, 15 years old, who underwent a bone marrow transplant. The patient was admitted to the pediatric intensive care unit (PICU) of a public hospital in Recife-PE, Brazil, presenting bone marrow aplasia, severe leukopenia, and thrombocytopenia. After 15 days, the patient developed right knee effusion. Synovial fluid and blood samples were analyzed at the Medical Mycology Laboratory of the Federal University of Pernambuco. Mycological diagnosis was based on the presence of hyaline septate hyphae on direct examination and the isolation of Fusarium oxysporum in culture, confirming the case of disseminated fusariosis. In vitro, the isolate showed fluconazole resistance and sensitivity to amphotericin B, anidulafungin, and voriconazole. Therapy with voriconazole in combination with liposomal amphotericin B led to an improved clinical response; however, due to underlying disease complications, the patient progressed to death.