Trichoblastoma is a rare benign biphasic, epithelial and mesenchymal, adnexal tumor recapitulating the hair bulb. It can be developed alone or rarely associated with nevus sebaceus of Jadassohn. Microscopically, it can mimic basal cell carcinoma. Awareness of their clinical, histological, and immunohistochemical characteristics is essential for proper pathological interpretation. This paper discusses a case of nevus sebaceus-associated trichoblatoma in a 13-year-old girl with histological features mimicking basal cell carcinoma.