Pilomatrixoma, also known as calcifying epithelioma of Malherbe, is a benign skin neoplasm that arises from hair follicle matrix cells. Pilomatrixoma is a common skin neoplasm in the pediatric population that is often misdiagnosed as other skin conditions. This study reviews an 11-year experience at a tertiary children’s hospital, examining the cause, clinical and histopathological presentation, management, and treatment outcomes of pilomatrixoma. A review of the pathology database at Children’s Hospital Los Angeles revealed 346 pilomatrixomas excised from 336 patients between 1991 and 2001. The hospital charts, pathology records, and plastic surgery clinic charts were reviewed with respect to variables such as sex, age at the time of presentation, clinical and histopathological presentation, preoperative diagnosis, management, recurrence, and treatment outcome. The main presenting symptom was a hard, subcutaneous, slowly growing mass. The preoperative diagnosis was accurate and consistent with the pathological diagnosis of pilomatrixoma in only 100 cases (28.9 percent). This entity should be considered with other benign or malignant conditions in the clinical differential diagnosis of solitary firm skin nodules, especially those on the head, neck, or upper limbs. The diagnosis can generally be made with a clinical examination. Imaging studies are not required unless symptoms or the location of the lesion warrants such diagnostic assessments. The treatment of choice is surgical excision, and the recurrence rate is low.