To report a rare case of an angioleiomyoma involving the orbit.

A 32-year-old male visited our clinic with pain and decreased visual acuity in the left eye, which had developed 1 month prior to admission. Best corrected visual acuity of the left eye had decreased to 0.7, and 3 mm of proptosis was observed. A 1×1 cm sized soft orbital mass was palpated on the lateral area of the left lower eyelid. Funduscopic examination of the left eye revealed multiple horizontal choroidal folds. On magnetic resonance imaging, a well-demarcated tumor was identified that showed hypointense signal intensity on a T1-weighted scan and hyperintense signal intensity on a T2-weighted scan. We performed anterior orbitotomy with an inferior conjunctival forniceal incision. The tumor was well encapsulated and easily dissected and could be completely removed. Histopathologic examination showed many vascular elements and intervascular fibrous stromas composed of spindle cells. The tumor was classified as angioleiomyoma.

Angioleiomyoma should be considered in the differential diagnosis of a well-circumscribed mass lesion involving the orbit.