In 1980, Rothko, Farmer, and Zeligman first described the disease entity "Superficial epithelioma with sebaceous differentiation" as a histologically distinct, rare, benign lesion. We report here the first Japanese case fitting this description. The patient is a 38-year-old Japanese woman, who had a small, yellow, flat-topped, well-defined plaque on her right upper eyelid. Histologically, the lesion was a multilobular, superficial, dermal tumor with numerous broad attachments to the overlying epidermis. The overall configuration was a plate-like proliferation of tumor cells with well-defined borders. The tumor cells were basically uniform basaloid cells with single or clustered sebocytes in the upper part of the tumor and multiple well differentiated sebaceous lobules in the middle and lower parts of the tumor. Multiple cystic spaces were formed in the lobules. Differential diagnosis of several tumors with sebaceous differentiation is discussed.