Pilomatricoma, although rare, is the second most frequent benign skin tumor in childhood. It is a hard, painless, slowly progressing subcutaneous tumor that is commonly located in the head-neck region, especially periorbital, and in the upper extremities. Histopathologic characteristics are distinctive. In this study, we performed a retrospective review of the records of our pediatric patients with pilomatricoma. We discuss herein the diagnosis and management of this uncommon skin tumor, and compare our experience with the previous literature. Pilomatricoma should be included in the differential diagnosis of superficial pediatric head and neck masses by any physician involved with pediatric tumors.