Dermatofibrosarcoma protuberans (DFSP) occurs most commonly on the trunk, affects all races, and often develops between the second and fifth decades of life. It is uncommon in childhood and is sometimes mistaken for a vascular lesion, as it often presents as a blue macule or small nodule. Review of the English literature revealed approximately 152 cases of DFSP developing before 16 years of age and only 19 claimed congenital cases. A case of congenital dermatofibrosarcoma protuberans is presented with a literature review and discussion of congenital and childhood presentations of the tumor.