Introduction: Fusarium species are hyaline saprophytic fungi that are frequently found in the soil, air, and water. They can cause severe systemic infections in immunocompromised patients. Clinical manifestations depend on the way of entry of the mold and host immune system status. The main ways of entrance are the airways, skin, and mucosal membranes. Disseminated fusariosis often occurs in patients with hematological disorders, patients with cancer, and solid organ transplant recipients. Case Presentation: Herein we report a case of Fusarium fungemia in a patient with adrenocortical carcinoma from Isfahan, Iran. The patient was a 41-year-old female with stage III adrenal cortical carcinoma. Despite antifungal therapy with liposomal amphotericin B, the patient passed away 6 days after admission. Internal transcribed spacer region sequencing applied for species identification and its sequence deposited in the GenBank (accession number: MK880379). Conclusions: Since the ideal strategies against invasive fungal infections remain uncertain and the mortality rate is high, we recommend primary prophylaxis with a broad-spectrum antifungal agent for vulnerable patients particularly those admitted to high-risk units such as oncology, hematology, and transplant units.