Pilomatrical carcinoma is a rare malignant tumor that originates from hair matrix cells. Pilomatrical carcinoma may arise de novo as a solitary lesion, or through transformation from its benign counterpart, pilomatrixoma. Differentiation between pilomatrixoma and pilomatrical carcinoma requires close histologic examination and often is difficult. Although uncommon, pilomatrical carcinoma has the potential to metastasize; therefore, prompt diagnosis and appropriate management is essential.