Acral pseudolymphomatous angiokeratoma of children (APACHE) is a new clinical entity that is characterized by angiomatous papules on the extremities. We report a case APACHE in a 14-year-old Japanese girl with asymptomatic red and violaceous papules and nodules on the ring finger of the left hand. Histological and immunohistochemical studies of our patient indicated that APACHE is a pseudolymphoma rather than an angiokeratoma.