An 11-year-old girl had a slowly enlarging, painful, pink-red tumor on her scalp of 1 month's duration. The lesion was composed of several, round tumor cell nests in the dermis. Each nest was made up of peripheral basophilic cells and central shadow cells or eosinophilic materials. Occasionally the nests, having central eosinophilic degeneration, became epithelial cysts, some of which showed acantholytic changes in their cyst wall. This seems to be the first case of an acantholytic variant of pilomatricoma.