We reviewed all occurrences of dermatofibrosarcoma protuberans in children under 17 years of age who were evaluated at Sainte-Justine Hospital, a tertiary-care pediatric center, between 1980 and 2002. The medical records and histologic features of all were reviewed. Eight patients were identified, three boys and five girls. The interval between apparent onset and diagnosis ranged from 3 months to 14 years. All lesions except one were removed by surgical excision with a margin of 1.5 to 3 cm including underlying fascia or by Mohs technique. Follow-up ranged from 2 to 15 years with median of 5 years. To date all the patients are alive and none has had a recurrence.