Papillon-Lefevre syndrome (PLS) is a rare genodermatosis of autosomal recessive inheritance manifesting as palmoplantar hyperkeratosis with periodontitis. A 4-year old Iranian girl was referred to the Mazandaran University Hospital, Sari complaining of well-demarcated, psoriasiform, yellowish, keratotic plaques over the skin of her palms and soles with transgrediens. For cutaneous lesions, she was treated with oral acitretin 0.5 mg⁄kg⁄day and topical keratolytic 5% salicylic acid in combination with 10% urea.