We report a 53-year-old man with a 2-year history of a violaceous indurated plaque on the shoulder. Although angiosarcoma was clinically suspected, histological examination revealed numerous lobules ('tufts') with cleft-like vascular lumina throughout the dermis and subcutaneous tissue. Tumour cells had no nuclear atypia and were positive for CD34, but almost negative for factor VIII-related antigen. These findings were compatible with a diagnosis of tufted angioma, or angioblastoma. We reviewed 41 cases reported in Japan and found that, although most patients presented during the first year of life (23/41), the condition does occur throughout childhood and adult life. Both sexes are affected equally and, contrary to some reports, it is unlikely that oestrogens have a pathogenic role.