We report a 13-year-old girl with an extensive bluish phlebectasia of the upper right arm and right side of the chest, which had been present since birth. There was no difference in length between the right (affected) and left (healthy) limbs, but the involved limb was thicker than the noninvolved limb. Magnetic resonance imaging showed distended veins with slow blood flow under the skin of the right limb. The veins inside the muscles of forearm were also involved. Histological examination of the bluish lesions revealed large phlebectasia showing distended veins without any proliferation of endothelial cells. The amount of elastin in the walls of these veins was decreased. The patient was diagnosed with Bockenheimer's syndrome. The characteristics of this rare syndrome are indicated and discussed.