Angiosarcoma is a rare and highly malignant neoplasm. Cutaneous angiosarcoma not associated with postmastectomy lymphedema is rare. There are only eight reported cases of development of cutaneous angiosarcoma in a lymphedematous extremity in a patient with preexisting malignancy other than breast cancer. This is the first reported case of development of cutaneous angiosarcoma in a lymphedematous extremity in a Hodgkin's disease survivor. A review of cutaneous angiosarcoma associated with lymphedema and a discussion of the pathogenesis of angiosarcoma are included.