We report about a 12 year old boy who was born with a linear epidermal nevus. This nevus extended from the umbilicus strictly along the midline to the symphysis and continued on the right leg caudally to the medial brim of the foot and the first and second toe. Changes of bone structure are seen at-x-ray examination of the right hip and leg. Vascular dysplasia with arteriovenous shunts and spinal angiomas were found by angiography in the vascular bed of the left iliac artery. The length of the legs differed due to hyperplasia of the left lower extremity and both feet resembled the "Friedreich-foot".