This article presents the case of an 11-year-old boy diagnosed with Papillon-Lefèvre syndrome (PLS), a rare autosomal recessive disorder. The patient exhibited symptoms such as palmoplantar keratoderma (PPK), gingivitis, and severe periodontitis, along with psoriasiform plaques. PLS is characterized by the combination of skin abnormalities and early-onset periodontal disease, caused by mutations in the *CTSC* gene. The article discusses the importance of early diagnosis, genetic testing, and multidisciplinary management to prevent tooth loss and improve the patient’s quality of life.