A 22 year-old woman presented with multiple soft, compressible, protuberant, bluish cutaneous lesions as well as firm, non-compressible, subcutaneous masses and varicose veins affecting the right upper limb of three years duration. There was atrophy of soft tissue of forearm by 2.5 cm. X-ray showed soft tissue densities, multiple phleboliths and hypoplastic forearm bones. Histopathological examination from cutaneous lesions revealed cavernous hemangioma. Adult onset cavernous hemangioma involving one upper limb and breast with multiple phleboliths and limb atrophy is a very unusual presentation of Klippel-Trenaunay-Weber syndrome.