Pilomatricoma is characterised as a common, slowly growing benign cutaneous tumour that appears generally within the first decades of life. The clinical diagnosis is frequently missed, especially by the paediatrician unfamiliar with these tumours. We present the experience gained in three European tertiary care paediatric centres with the treatment of pilomatricoma and also current data on the aetiology, clinical presentation and management. A retrospective study was carried out in 83 patients suspected for pilomatricoma during a 7-year period (1996-2002) at the departments of Paediatric Surgery of the Children’ s University Hospital “ Federico II” , Naples, Hospital “ San Bortolo” , Vicenza and “ Aghia Sophia” Children’ s Hospital, Athens. The age range was from 10 months to 17 years, median age 8 years. All patients were treated by surgical excision and all specimens were examined by histopathological assessment. The follow-up varied from 5 months to 6 years. The correct diagnosis was made preoperatively in 68 patients (82% ). The female/male ratio was 2:1. The sites of occurrence were the head (47.5% ), especially in the periorbital region, the neck (9% ), the upper limbs (35.5% ), the inferior limbs (4% ) and the thorax (4% ). Each patient exhibited a single pilomatricoma except for two patients who had multiple lesions (2.4% ). One of them had Steinert disease (myotonic dystrophy). No recurrences were observed during the follow-up period. Conclusion: Pilomatricoma is one of the most common cutaneous adnexal neoplasms in children. Surgical excision including clear margins and its overlying skin in most cases is the treatment of choice. The recurrence as well as malignant evolution is rare. Pilomatricoma is characterised as a common, slowly growing benign cutaneous tumor that appears generally within the first decades of life. The clinical diagnosis is frequently missed, especially by the pediatrician unfamiliar with these tumors. We present the experience gained in three European tertiary care paediatric centers with the treatment of pilomatricoma and also current data on the aetiology, clinical presentation and management. A retrospective study was carried out in 83 patients suspected for pilomatricoma during a 7-year period (1996-2002) at the departments of Pediatric Surgery of the Children ’s University Hospital “Federico II”, Naples, Hospital “San Bortolo”, Vicenza and “Aghia Sophia” Children’s Hospital, Athens. All age were from 10 months to 17 years, by surgical excision and all specimens were examined by histopathological assessment. The follow-up varied from 5 months to 6 years. The correct The female / male ratio was 2: 1. The sites of occurrence were the head (47.5%), especially in the periorbital region, the neck (9%), the upper limbs (35.5%), the inferior limbs (4%) and the thorax (4%). Each patient featured a single pilomatricoma except for two patients who had multiple lesions (2.4%). One of them had Steinert disease (myotonic dystrophy). No recurrences were observed during the follow-up period. Conclusion: Pilomatricoma is one of the most common cutaneous adnexal neoplasms in children. Surgical excision including clear margins and its overlying skin in most cases is the treatment of choice. The recurrence as well as malignant evolution is rare.