| Summary: | Trichilemmal carcinoma (TC) is a rare malignant adnexal neoplasm, falling under the umbrella of tumors with follicular differentiation generally presenting as papulonodular growth with surface ulceration over sun-exposed skin. Histologically, it mimics tumors such as basal cell carcinoma, sebaceous carcinoma, squamous cell carcinoma, and malignant proliferating trichilemmal tumors. As there is a difference of opinion regarding the histopathological criteria for TC, a definitive diagnosis should be confined to cases having the majority of histomorphological findings with immunohistochemical corroboration to help define the clinicopathological behavior of this disease. Rarity, indolent clinical course in spite of local aggressiveness and a good survival rate postexcision further undermine the importance of this unique entity.
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