Herein, we report a case of the hyperkeratotic variant of porokeratosis Mibelli. A 58-year-old woman presented with two brownish verrucous plaques on the buttock for 20 years, and extremely hyperkeratotic verrucous nodules and brownish macules on the right foot for 1 year. Histopathological findings of the buttock were typical of porokeratosis. However, histopathological findings of the right foot were atypical for porokeratosis, including extreme hyperkeratosis and an increased number of and unusual sized cornoid lamellae.