Olmsted syndrome is a rare congenital mutilating palmoplantar keratoderma associated with periorificial keratotic plaques. Treatment options include topical keratolytics, systemic retinoids, and debulking procedures. Full-thickness excision of hyperkeratotic plaques followed by skin grafting has been reported in the medical literature, although long-term results have not been evaluated. We present two cases of Olmsted syndrome with severe palmoplantar keratoderma treated with excision and skin grafting, along with long-term clinical results 11 years (patient 1) and 6 years (patient 2) following the initial surgery.