A case of hemangiectatic hypertrophy and congenital phlebariectasis is described. The cardinal features of this case are extensive vascular nevus, hypertrophy of the right upper extremity and varicose veins of the right lower extremity. In this case the clinical and radiologic findings were at variance with studies of oxygen saturation. In the second case it is shown that congenital aplasia of venous structures can lead to a picture almost similar to the hemangiectatic hypertrophy of Parkes Weber.