Proliferating trichilemmal cyst (PTC) is a rare but morphologically distinctive tumor that usually occurs in the scalp of elderly women. Furthermore, only 30 well-documented examples of so-called malignant PTC have been reported. Alternatively, some authors have recently proposed that PTC is squamous cell carcinoma. We describe a case of PTC with focal invasion that was indistinguishable from squamous cell carcinoma in areas, and we review the literature. This debate should be considered in PTCs to provide adequate treatment (wide local excision) and follow-up (long term), particularly in large and long-standing lesions.