Kaposi’s sarcoma localized to the penis with striking lymphoedema is extraordinary. We report a middle-aged Haitian man who was human herpesvirus-8 seropositive, without evidence of immunosuppression or human immunodeficiency virus infection. He was first seen with Kaposi’s sarcoma of 6 months duration localized to his penis, preceded by a 3-year history of chronic penile lymphoedema. His tumour regressed completely after radiotherapy. We propose that chronic lymphoedema in this patient predisposed to the development of Kaposi’s sarcoma.