Abstract: A 27-year-old female presented with scalp plaque for 2 months. Histopathology showed that the tumor grew horizontally and vertically, involving epidermis, dermis and superficial adipose tissue. Cellular atypia and pathological mitotic phases were noted. Then extensive resection of the tumor was performed. Histopathology showed that the tumor cells in the epidermis grew like paget's disease, and there were still tumor cells remaining. Immunohistochemistry: EMA (+), AR (-), CAE (-), ER (-) of tumor cells, the ratio of Ki67 positive cells was 30%. The diagnosis of sebaceous carcinoma was made. Resection should be performed again, supplemented by radiotherapy or chemotherapy.

Key words: extraocular sebaceous carcinoma, extensive resection