Background: Papillon-Lefevre Syndrome (PLS) is a rare genetic disorder, characterized by palmoplantar keratosis, aggressive periodontitis and premature edentulous primary and permanent dentition at a very young age. Additional symptoms and findings associated with PLS may include frequent pyogenic skin infections, abnormalities of the nails, and excessive perspiration. Case report The patient, who visited in the Central dental hospital was 3.6 years old. Pediatric dentist confirmed the diagnosis “PLS and chronic traumatic tongue ulcer” based on anamnesis and objective examination. Dental and tongue ulcer treatment was done once a week for 2 months. The recall system was carried out by 3 month intervals oral examination and 6 month intervals radiographic examinations under team of pediatric dentist, pediatrician and immunologist.