Trichilemmal cyst (TC) is an adnexal skin tumor that usually occurs on the scalp. There is a female dominance in this tumor. Proliferating pilar tumors or cysts arise from this lesion, and the clinical differential diagnosis from TCs must be taken because of aggressive behavior of proliferating pilar tumor. Trichilemmal cyst usually occurs in hair-bearing areas such as the neck, trunk, groin, and others. In this article, we report a case with giant TC involving the neck region.