Background:Dermatofibrosarcoma protuberans is a rare mesenchymal malignancy in childhood and adolescence. The tumor is characterized by dermal spindle cell proliferation with infiltration of subcutaneous tissue, expression of CD34, and a specific fusion of the platelet-derived growth factor beta with the collagen type 1alpha1 gene.Main observation:We observed a 10-year-old girl with a medaillon-like, asymptomatic plaque on the chest that was diagnosed as DSFP. The tumor was completely removed by delayed Mohs surgery. Follow-up so far has shown a complete response.Conclusions:The prognosis of dermatofibrosarcoma protuberans in children is excellent as long as early diagnosis is followed by complete excision with Mohs surgery as a golden standard.