Extraocular muscle lipoma is a rare benign mesenchymal tumor of the orbit. We report a case of a 37-year-old woman who presented with chronic progressive proptosis and inferior globe displacement of left eye. External eye examination revealed a yellowish mass at the superior bulbar conjunctiva. Magnetic resonance imaging showed a well-circumscribed mass confined in the superior rectus muscle belly and tendon with a fat signal. Debulking surgery was performed using the transconjunctival and vertical lid split approach. A pathological study demonstrated matured adipose tissue cells encapsulated by a thin layer of fibrous tissue, in addition to the chronic non-specific inflammation of the tenon capsule tissue sample. Histopathological findings of the mass were consistent with a well-circumscribed intramuscular lipoma. The symptoms of the patient were significantly improved 3 months after surgical and short-course systemic steroid treatments. However, long-term surveillance is needed.