Leiomyoma is a rare, benign neoplasm of the nipple. We report the case of a patient with a leiomyoma arising from her left nipple who presented with persistent nipple pain and tenderness which led to the eventual complete excision of her left nipple and areola complex. The clinical characteristics, gross and microscopic pathologic findings, and management of this lesion are discussed. Surgical management should be tailored primarily to the control of symptoms. Clinicians should be aware that this is a rare, but potential cause of chronic persistent pain and swelling in the region of the nipple-areola complex of the breast.