Alveolar soft part sarcoma (ASPS) is a very rare type of soft tissue sarcomas which usually occurs in the limbs and trunk. A 25-year-old woman presented with proptosis and redness of the right eye for 8-month. She suffered from severe right upper lid edema, conjunctival chemosis, downward displacement of the globe, and proptosis. Radiological imaging was nonconclusive. Histopathological evaluations confirmed ASPS. The patient underwent exenteration as a lifesaving procedure. At 16-month follow-up, the patient is stable without any signs of recurrence or metastasis. We report an extremely rare case of ASPS occurring in the superior rectus muscle. Few orbital ASPS cases have been reported in the literature. A literature review of orbital ASPS was done to shed lights on the diagnosis and management of this rare tumor.