Bednar tumor is a rare pigmented variation of dermatofibrosarcoma protuberans, 
present in 1 to 5% of all patients with dermatofibrosarcoma protuberans. No 
significant clinicopathologic differences exist between Bednar tumor and 
conventional dermatofibrosarcoma protuberans apart from the presence of 
scattered nonneoplastic pigmented dendritic cells in the former. Although most 
dermatofibrosarcoma protuberans occur in adults, they may be rarely present at 
birth. Fanconi anemia is a genetically heterogeneous chromosomal instability 
syndrome, characterized by multiple congenital anomalies, progressive bone 
marrow failure, and a predisposition to malignancy. We describe here a patient 
with Fanconi anemia who had a congenital Bednar tumor. To our knowledge, this is 
the first such patient described with both dermatofibrosarcoma protuberans and 
Fanconi anemia.