The Olmsted syndrome is a rare congenital disorder with palmoplantar keratoderma and hyperkeratotic periorifical plaques. Only 19 cases were reported in the literature. Other cutaneous and organic findings have been reported with different frequencies.A 17-year-old girl presented with genital, perigenital and perianal hyperkeratotic plaques extending to the thighs, palmoplantar keratoderma and nail dystrophy. A small erythematokeratotic plaque was localized below the lower lip. Improvement of the palmoplantar and genital lesions was noted after one month of treatment with 25 mg acitretine daily. Further substantial improvement of the palmoplantar and ungueal lesions was observed during the next 7 months. However, discrete erythematous perianal and perigenital lesions persisted in these areas 2 years later.Efficacy of retinoids in the Olmsted syndrome is controversially discussed in the literature. However, acitretin seems to be the treatment of choice of this syndrome in adults.