Acral pseudolymphomatous angiokeratoma of children (APACHE) is a disease comprised by a dense dermal infiltrate of B-lymphocytes and T-lymphocytes in which prominent blood vessels with plump endothelium are found. In the past, the lesion was interpreted as a variant of angiokeratoma, a vascular malformation, or a nevus. Currently, most authors consider it to be a type of pseudolymphoma with prominent blood vessels. The latter express CD34 and D2–40, while they lack the expression of Glut-1. The expression of Wilms tumor-1 (WT-1) by APACHE has not yet been studied. In this report, we present a case of APACHE on the right foot of a 4-year-old boy and demonstrate immunoexpression of WT-1 by the blood vessels of the lesion. We also performed serial sections and demonstrated that the WT-1+ vessels with prominent endothelium were D2–40−.