RESUMEN
A 71-year-old male presented with a history of noticing gradual painless progressive proptosis along with a pinkish mass seen on the outer aspect of his left eyeball. Orbital CT revealed a fairly defined homogenous lesion, abutting the left eyeball and involving the lateral orbit reaching almost till orbital apex. Histopathology analysis aided with immunohistochemistry after an incision biopsy was consistent with leiomyosarcoma. Positron emission tomography scan ruled out presence of any distant primary or metastatic tumor. He underwent eyelid sparing total orbital exenteration with confirmed tumor free soft-tissue margins. He is doing well at 5 months of follow-up with no sign of local recurrence or distant metastasis. Primary orbital leiomyosarcoma is a rare tumor. It is mainly found in older woman; it is even rarer in males. Complete surgical excision with tumor free margins is the recommended treatment. Histopathology and immunohistochemistry are valuable in diagnosis and management.
