ABSTRACT Purpose This article presents a simple classification of lymphedema of the external genitalia, which is useful for selecting the appropriate therapy, and evaluates our experience with the various therapeutic options used to treat this disorder. Materials and Methods The literature was reviewed and the records of patients treated for the disorder were analyzed. Results A convenient classification of the disorder divides cases into congenital and acquired. Therapy is primarily dependent on whether the disease is self-limited and whether there has been any pathological change in the skin, lymphatics and subcutaneous tissue. For self-limited diseases in which no permanent pathological sequelae occur conservative therapy is appropriate. For most chronic conditions a surgical procedure is required. Excisional techniques are most effective for severe forms of the disease. In select cases subcutaneous tissue excision with preservation of the overlying skin is appropriate. However, for most patients excision of the skin and subcutaneous tissue with split-thickness grafting is most effective. Conclusions When patients with lymphedema of the external genitalia require surgery and are properly selected for the appropriate procedure, the functional and cosmetic results are excellent and patient rehabilitation is likely.