PurposeTo report a case of gastrointestinal sarcoma with leiomyosarcomatous differentiation metastatic to the eyelid.MethodsClinical data including a comprehensive ophthalmologic examination, histologic findings, a bone scan, and hospital records were reviewed.ResultsA 56-year-old woman with a history of esophageal cancer had a rapidly growing lesion on her right upper eyelid that was initially treated as a chalazion. A biopsy specimen of the lesion was consistent with a gastrointestinal sarcoma with leiomyosarcomatous differentiation. The esophageal tumor was reclassified after histopathologic evaluation of the eyelid specimen. Bony metastasis developed soon after excision of the eyelid lesion. The patient died 3 months after proper diagnosis of the eyelid lesion.ConclusionsThis is a rare case of a metastatic leiomyosarcoma of the eyelid. To our knowledge, only two other cases of eyelid leiomyosarcoma have been described in the literature. The importance of correct histopathologic diagnosis of eyelid lesions is underscored in this case report.