[Stewart-Bluefarb syndrome (kaposiform arteriovenous fistula with bone changes)]

In a 14-year-old female patient with arteriovenous shunts (Stewart-Bluefarb syndrome), fist-sized cutaneous swelling, venous dilatation, blue-brown pigmentation and painful subcutaneous fingertip-sized nodules were noted on the right lower thigh. In addition to an arteriovenous malformation, bone hypertrophy, bone atrophy and after bone defects were observed in the involved area on X-ray examination. Histological examination revealed increased vascularity and vascular cavernous structures with endothelial swelling and extravasation of erythrocytes, but no malignant changes.