We presented a case of Kaposi's sarcoma that occurred in the external ear of a 36-year-old Caucasian man. He was otherwise healthy without a history of any predisposing factors. He had a nodular lesion in the left ear, of three-month duration. The lesion was excised completely. Histologic and immunohistologic findings were consistent with a diagnosis of Kaposi's sarcoma. Serologic analyses were negative for anti-HIV antibody and anti-cytomegalovirus IgM and IgG and blood count was normal. Differential count of leucocytes and immunoglobulin electrophoresis were normal. During a two-year follow-up, no recurrences, development of new lesions, or HIV seroconversion were detected. To our knowledge, this case is the first to report a solitary lesion of Kaposi's sarcoma occurring in the helix of the ear in a healthy young patient
