Pilomatrix carcinoma (synonyms, matrical carcinoma or malignant pilomatrixoma) is a rare malignant neoplasm derived from the hair matrix first described in 1980. This neoplasm can exhibit local aggressive behavior and distant metastasis. Most pilomatrix carcinomas occur on the head and neck of elderly individuals with a predilection for males (M:F 5:1). Pilomatrix carcinoma is often clinically misdiagnosed as a sebaceous cyst and histologic difficulty can occur in differentiating this entity from the benign entity pilomatrixoma. The objective was to describe a case of pilomatrix carcinoma encountered in a Mohs micrographic surgery practice. We present the first case of this lesion treated by Mohs surgery. A case report and literature review are presented. Pilomatrix carcinoma is a rare malignant variant of pilomatrixoma. Given the rarity of this lesion there are no well-defined standards for surgical management. Wide local excision has been recommended given the high rate of reoccurrence. Mohs micrographic surgery may provide optimal treatment of this neoplasm given the ability to have 100% margin control.