Papillon Lefevre syndrome is presented in the cases of two female patients of the ages of 7 and 9, who exhibited all typical symptoms of the disease. Microbiological and histopathological studies were done and treatment provided. Actinobacillus actinomycetemcomitans, which is suspected as a pathogenic factor in the disease was identified as well as some other gram negative microorganisms and an antibiogram was performed in which amoxycillin plus clavulanic acid was most effective. Histopathological investigation also confirmed the presence of gram negative bacteria. Granular cell infiltration was predominant in the surface epithelium. Prosthetic appliances were provided for the patients after mechanical and chemical plaque control. In addition to this, antibiotics (amoxycillin plus clavulanic acid) were prescribed every six months. No tooth loss was observed in both patients after more than two years follow-up period. At the moment only one patient is under review and because she is uncooperative, mild periodontal inflammation is still present around the teeth which erupted before the antibiotic regime, but not in the other teeth.