A 56-year-old Asian woman presented with an upper eyelid mass. The lesion was exposed after eversion of the eyelid revealing a thickened tarsus with yellowish areas. Working diagnosis was sebaceous carcinoma. Biopsy was performed. Histopathological studies showed a mycotic eumycetoma with Splendore-Hoeppli phenomena and-microbiologic cultures grew Scedosporium apiospermum. The patient was started on voriconazole 200 mg po bid with adequate serum levels. A complete response was observed after 18 weeks of voriconazole therapy. To the best of our knowledge, this is the first published case of S. apiospermum eumycotic mycetoma of the eyelid. It is important to consider mycotic infection in the differential diagnosis of eyelid tumors even in healthy patients.