Papillon-Lefevre syndrome (PLS) is a rare recessive autosomal disease which occurs with palms and soles hyperkeratosis as well as primary and permanent teeth periodontal loss. Recently, it has been proved that a mutation at Cathepsin gene C is the genetic cause of PLS. All primary and permanent teeth of the patients are lost at ages 2 or 3 because of serious alveolar bone destruction. This paper presents a complete case of prosthetic treatment of an 8-year old girl with PLS.