Lymphedema, which can be seen as primary and secondary, is caused by a failure of lymph drainage resulting in chronic tissue swelling.
There are two types of primary lymphedema: hereditary and nonhereditary. The hereditary type has two subtypes: early and late onset. The most critical complication of chronic lymphedema is the development of lymphangiosarcoma. This generally develops on a base of secondary lymphedema, caused by radiotherapy and mastectomy which has been performed in the treatment of breast cancer. It rarely develops on the leg. Here, a 41 year-old male with late onset hereditary primary lymphedema in the leg on which lymphangiosarcoma has developed is presented. Moreover, his daughter was also diagnosed with late onset hereditary primary lymphedema. This case is presented due to the fact that lymphangiosarcoma on the leg is rarely seen.
