Pilomatricoma, calcifying epithelioma of Malherbe, is a benign skin neoplasm of hair matrix origin that is typically occurred in the head and neck region. It usually presents as a superficial, firm, solitary, slow-growing, and painless mass of less than 3 cm in diameter and most often occurs in young age under 20 years. The tumor does not express an aggressive behavior and surgical excision is the treatment of choice. The purpose of this article is to present a case of pilomatricoma of the preauricular region and to review the literature regarding pilomatricomas of the head and neck region.