Atypical Fibroxanthoma in a 13‐Year‐Old Guatemalan Girl with Xeroderma Pigmentosum

Xeroderma pigmentosum ( XP ) is a rare, autosomal recessive disease involving a defect in DNA repair leading to the premature development of numerous aggressive cutaneous malignancies. Although atypical fibroxanthoma ( AFX ) is a neoplasm typically found in the setting of extensive sun exposure or therapeutic radiation, AFX s are rarely associated with children with XP . We report the case of a 13‐year‐old Guatemalan girl with the XP type C variant who developed one of the largest AFX s reported on a child's finger.