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To review the clinical features of pilomatrixomas of the eyelids and eyebrows in children and adolescents.The authors reviewed the medical records of 95 patients, all of whom were 20 years old or younger and had the histopathologic diagnosis of pilomatrixoma made from 1976 through 1995. Special attention was given to that subgroup of patients with tumors involving the eyelids and eyebrows. Data analyzed included age at diagnosis, patient sex, location and size of tumor, preoperative diagnosis, and clinical description of tumor.Eleven (12%) of 95 patients had lesions involving the eyelid or eyebrow. Six of those patients (55%) were female. Eight patients (73%) were 10 years old or younger, and three were 11 to 20 years old. The tumor occurred on the upper lid or in the brow in eight patients, the lower eyelid in two patients, and the medial canthus in one patient. Tumor diameter was 1 cm or less in all 11 patients. Pilomatrixoma was included in the preoperative differential diagnosis in only four of the 11 patients. The most common misdiagnoses were epidermoid and dermoid cyst. Three patients had pink or blue discoloration of the overlying skin, two had visible yellow-white patches in the tumor, and two had dilated vessels on the tumor surface.Pilomatrixoma is an uncommon tumor that can occur on the eyelids and in the brow region of children and adolescents. It is commonly misdiagnosed preoperatively as an epidermoid or dermoid cyst. Distinctive clinical features, however, suggest the correct diagnosis.