Olmsted syndrome is a rare congenital entity characterized by combination of symmetrical, sharply-defined palmoplantar keratoderma with flexion deformities of the digits, periorificial keratosis, perianal involvement, onychodystrophy, and variable leukokeratosis. Herein, we report two new related male patients--the third familial cases of Olmsted syndrome--one with the full-blown spectrum of the syndrome, and the other with early signs and symptoms of the disorder.