Background: Osteochondromyxoma is an extremely rare benign tumor. Few cases have been described in the literature. Patients with Carney complex may present with this tumor. Case presentation: We report a case of a nasal osteochondromyxoma in a 21-year-old female patient who presented with progressive nasal congestion, anosmia and episodes of self-limiting epistaxis. Magnetic resonance imaging and computerized axial tomography scans were performed which demonstrated a large tumor occupying the nasal cavity. Endoscopic sinus surgery was performed resulting in complete resection of the tumor. No recurrence has been observed at 24 months following endoscopic sinus surgery. The patient has also recovered olfaction. Conclusion: Although osteochondromyxomas are extremely rare, it should be considered as a differential diagnosis for nasal tumors, and surgical resection should be completed. A multidisciplinary assessment is recommended as it is a major criterion for the diagnosis of Carney complex.

Keywords: osteochondromyxoma; Carney's complex; nasal tumor; paranasal sinuses, case report