Pilomatricoma is a skin appendage tumor that frequently involves the skin of the head, neck, and upper extremities in young children. In this prospective study, we present and discuss the characteristics of our patients with pilomatricoma. During the years 1984 to 1994, 15 children (9 girls and 6 boys) with pilomatricoma were admitted to our service. Their ages ranged from 3 to 13 years (mean age 9 years). All of the lesions of these patients were excised totally. On histopathologic examination, calcification was found in all of the specimens, No invasion or malignancy was observed by light microscopy, All patients have been followed up periodically by the same surgeons. Interestingly, we found two familial occurrences and four multiple occurrences. Although our series was too small for accurate statistical evaluation, multiple and familial occurrences of pilomatricoma (26.7% and 13.3%, respectively) were higher than in previously reported series.