Papillon- Lefevre Syndrome (PLS) is a rare, autosomal recessive disease with no known etiological factor, which reveals loss of function mutations affecting both the alleles of the cathepsin- C gene. The disease is characterized by palmo-plantar keratoderma and early juvenile periodontitis affecting both primary and permanent teeth. Due to the severe alveolar bone loss both in deciduous as well as permanent dentitions, teeth are exfoliated within two or three years after eruptions. By the age of 15 or 16, patients are usually edentulous except for the third molars. These teeth are also lost a few yeas after they erupt.No treatment has been shown to be effective for keeping teeth. Prognosis for prevention of teeth loss is hopeless. Available treatment plans such as stabilization of the alveolar bone height improve the prognosis for a lifetime of complete denture use.In this article, a- 12- year old girl living in Bandar Abbas who has been referred to Oral Medicine Department of Shiraz Dental School for further evaluation, diagnosis and proper treatment plan is reported.Key words: Papillon- Lefever syndrome, Palmo plantar hyperkeratosis, early periodontitis