Introduction: Angioleiomyoma is a benign soft tissue tumor of smooth muscle origin with a vascular component and is an uncommon form of leiomyoma. Angioleiomyoma presenting in the nasal cavity is exceedingly rare and there are 68 reported cases in the literature worldwide. We present a case of angioleiomyoma of the nasal septum and review its diagnosis and treatment. Study Design: Case report and Literature Review. Methods: The medical records of a 69-year-old patient with an angioleiomyoma of the nasal septum were reviewed. The PubMed database was searched for literature describing anglioleiomyoma of the nasal cavity using the key words “angioleiomyoma” with “nasal cavity,” “nasal septum,” “nose,” or “sinus.” Results: A 69-year-old female ...