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Introduction: Sebaceous cell carcinoma is an aggressive tumor arising from the sebaceous glands. It is relatively rare tumor and seen almost exclusively on the eyelids (75%). Despite the high concentration of sebaceous glands over head and neck region, true neoplasm, i.e. sebaceous cell adenoma and carcinomas are infrequent. It accounts for just 0.2-0.7% of all eyelid tumors in USA and very few cases that have originated in areas other than the eyelids have been reported. Case Report: A 70-year-old male presented with complaints of rapidly growing swelling (3x4 cm), on the right post auricular region, since one and half months. The patient developed discharging ulcer over the swelling associated with progressive tinnitus and hoarseness of voice, since last twenty days. Fine needle aspiration cytology (FNAC) was advised, which suggested the diagnosis of sebaceous cell carcinoma and excision biopsy was done. Histopathological examination of excised tissue confirmed the diagnosis. Conclusion: Extraorbital sebaceous cell carcinoma is an aggressive and invasive malignancy. It clinically mimics other diseases and is difficult to diagnose. Hence, an accurate and prompt diagnosis is crucial because of its fulminant course, serious associations with Muir-Torre syndrome and high potential for regional and distant metastasis.

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