Inflammatory myofibroblastic tumor (IMT) is a tumor composed of differentiated myofibroblastic spindle-shaped cells. It occurs in the soft tissues of the abdomen and lungs, and is very rare in the sphenoid sinus. The diagnosis depends on histopathology and immunohistochemistry, and is easily misdiagnosed. Although metastasis is rare, the recurrence rate is high. Surgical resection is the treatment of choice, and where complete resection is difficult, radiation therapy, hormonal therapy, or molecular targeted drug therapy can be administered. We report a rare case of IMT with a primary origin in the sphenoid sinus, which was entirely resected by nasal endoscopy and confirmed by histological examination.

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