Steatocystoma is a cyst that originates embryologically from hair follicles and usually occurs on the face, chest, back and axillae as multiple lesions and rarely occurs as a solitary lesion. We experienced a case of steatocystoma simplex that developed in the orbit and present our case with a brief review of the literature.

A 44-year-old male presented with a history of palpable mass in the right inferomedial periorbital area that had been slowly growing for a few months. Physical examination revealed a non-tender, soft and round mass. Magnetic resonance imaging of the orbit showed 2.0 × 2.0 × 2.0 cm-sized mass in the right inferomedial orbit. We performed excisional biopsy of the orbital mass using transconjunctival approach. The pathological diagnosis was steatocystoma. There was no local recurrence during the postoperative follow-up.

We experienced a rare case of steatocystoma simplex that developed in the orbit. Base on our results, steatocystoma simplex should be considered when diagnosing an orbital mass.