To report a rare case of recurrent alveolar soft-part sarcoma (ASPS) with concurrent involvement of the orbit and multiple sites of the body that was removed successfully by surgery.

A 37-year-old woman presented with diplopia at the left lateral gaze and proptosis of the left eye. Two years earlier, the patient had a mass excision of the right gluteus maximus and the left orbit, and ASPS was histopathologically diagnosed at that time. In addition, the patient had been treated with chemotherapy and radiation therapy. On radiologic examination, recurrent tumor of the left orbit was found and surgically removed. The histopathologic examination showed that tumor cells were arranged in an alveolar pattern divided by fibrous septa and contained abundant granules in the cytoplasm, typically consistent with ASPS. Postoperatively, the symptoms of diplopia and proptosis improved.

ASPS can occur and recur in the orbit as well as systemically. In addition, at the time of surgical removal of ASPS in the orbit, the surgeon should be particularly cautious of massive bleeding.