Acral pseudolymphomatous angiokeratoma of children (APACHE) is a recently recognized, rare clinical entity. We report a case of APACHE in a 9-year-old Japanese girl with a dark red, linear plaque on the posterior area of the right lower leg. Histopathologic findings revealed further evidence that APACHE was a cutaneous pseudolymphoma rather than a vascular neoplasm because of the presence of distinct primary and secondary lymphoid follicles, and thick-walled, long blood vessels lined with prominent plump endothelial cells similar to high endothelial venules in the paracortical area of the lymph node. In addition to previous descriptions, the patient presented with a distinct linear lesion. Vacuolar alteration of the basal cell layer and scattered eosinophilic bodies in the epidermis were observed, which suggested a lichenoid tissue reaction. We believe that APACHE might show a distinct linear pattern accompanied by a lichenoid tissue reaction. (J Am Acad Dermatol 2003;48:S15-7.)