Introduction: Invasive fungal sinusitis is a potentially lethal infection in immunocompromised patients, with a reported incidence of around 2%. Neutropenia due to aplastic anemia or secondary to chemotherapy is the main cause of acute invasive fungal sinusitis. Case Presentation: We present the case of a 6-month-old boy with hemophagocytic syndrome. During the follow-up for fever and pancytopenia at the medical faculty hospital of Hacettepe University, Ankara, Turkey, the patient developed discoloration and black crusting of the nasal columella. Antifungal medical treatment and biopsy were recommended owing to the suspicion of invasive fungal infection. The premaxilla, nasal columella, and nasal cartilages were necrotic. Medical and surgical treatments were performed to prevent the spread of infection. After controlling the underlying immunosuppression, nasal reconstruction was advised. Conclusions: Invasive fungal sinonasal disease is a rare clinical phenomenon in immunocompromised patients. Although it is associated with high mortality rates, it can be successfully managed with a combination of surgical and medical treatments.