Congenital Dermatofibrosarcoma Presenting as an Atrophic Plaque

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Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma that most commonly occurs on the trunk of adult patients. However, DFSP can present congenitally, and this rarity may lead to misdiagnosis or late diagnosis. The authors present a case of a congenital DFSP that was not diagnosed until the patient was 17 years old. Her tumour was successfully treated with Mohs micrographic surgery. It is important to have DFSP on the differential for atrophic plaques or violaceous to red-brown nodules, even at a young age.