Background: Papillon-Levefre Syndrome (PLS) is a rare genetic disorder with prevalence of 1-4 cases per millions of birth, characterized by the presence of palmar-plantar hyperkeratosis and rapid periodontal destruction of both the primary and secondary dentition. Purpose: This clinical report described such a rare condition with special attention on its diagnostic characterization, various treatment options and Prosthodontic rehabilitation. Case: Five years-old boy accompanied by his mother came to Pediatric Dentistry Specialist Clinic, Universitas Airlangga Dental Hospital - Surabaya with the chief complaint of gradually loss of upper and lower teeth since the patient was 3 years old. Case Management: Removable partial denture was chosen because it was considered as an appliance that can recover the function of mastication, speech and aesthetic patient. Removable partial denture was designed to replace 51, 52, 53, 54, 55, 61, 62, 63, 64, 65, 72, 73, 74, 75, 82, 83, 84 and 85 with retention on 16, 26, 36 and 46, and anterior bite riser on 31 and 41. Conclusion.Removable partial denture is the treatment of choice for children with PLS who are still in the growth period because it can recover mastication and aesthetic functions, have a good aesthetic, and easy maintenance that could done by the child.

The most common symptoms of Williams-Beuren syndrome (WBS) are heart defects and unusual facial features. The objective of this case report was to highlight the preparation of comprehensive dental care for this syndrome. A seven-year-old boy came with multiple dental caries as a chief complaint. Parents reported a history of corrected tetralogy of Fallot (ToF) three years ago. The routine medical check-up was carried out annually. Based on the typical signs and symptoms, a diagnosis of William-Beuren Syndrome was made. Comprehensive dental care under general anesthesia on a patient with a history of heart defect and multiple dental caries was the best choice and exhibits a high success rate. Extraction treatment was carried out to avoid the risk of endocarditis infection, restoration to prevent the progressivity of caries, and space maintainers to restore function and aesthetics.