Background: Papillon-Lefèvre syndrome (PLS) is considered a rare syndrome, which is characterized by the presence of palmar-plantar hyperkeratosis and aggressively progressing periodontitis that finally leads to premature loss of both deciduous and permanent teeth. Case report: A four-year-old Egyptian boy presented with a maternal complaint that her child suffers from early loss of many teeth, presence of loose teeth along with an asymptomatic swelling related to the upper anterior area. The patient was diagnosed with PLS. A symptomatic management and prevention program was followed and the swelling was excised; afterwards diagnosed as peripheral ossifying fibroma. Conclusion: Early recognition and intervention for patients with PLS is essential to avoid the threat of being edentulous if left unmanaged.