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Papillon-Lefevre Syndrome is a rare autosomal recessive disorder. This syndrome accompanied by palmoplantar hyperkeratosis and severe periodontal destruction of primary and permanent teeth. The teeth erupt normally but due to the severe alveolar bone loss both in deciduous as well as permanent dentitions, these teeth are exfoliated within two or three years after eruptions and by the age of 15 or 17. Patients are usually edentulous. Due to periodontal disease, the dentists are often the first ones who diagnose the syndrome. A 15-year-old girl was referred to dental clinic complaining of permanent teeth mobility. All patient’s teeth except 13, 14, 17, 23, 27, 37, 43, 44 and 47 had been extracted. The third molars were impacted. Patient has advanced periodontal disease and all teeth have mobility. There was hyperkeratosis at the palms and soles. The teeth were extracted and treated with complete denture. Early diagnosis of the papillon-lefevre syndrome can help to preserve teeth. Dental treatment included extraction of all deciduous teeth, professional prophylaxis, conventional periodontal therapy, systemic antibiotics, oral retinoid, complete dentures and implants.