We report a rare case of a lacrimal sac angioleiomyoma. A 56-year-old woman complained of pain in the right medial canthal region over a period of 2 years. There were no complaints of epiphora or ocular infection, and no visible or palpable masses in the medial canthal region. Computed tomography scan revealed a solid tumor of the lacrimal sac expanding to the nasolacrimal duct and protruding under the inferior turbinate. The tumor was removed by external dacryocystectomy combined with endonasal, endoscopic anterior turbinectomy, and nasal mucosal resection. Histological and immunohistological findings were consistent with an angioleiomyoma of the venous type. There was no recurrence of the tumor at the three-year follow-up. Angioleiomyomas should be included in the differential diagnosis of lacrimal sac tumors. The definitive diagnoses rely on histology and immunohistological reactions. The treatment is complete surgical resection.