The Papillon-Lefevre syndrome (PLS) was first described by Papillon and Lefevre in 1924. It is an autosomal recessive disorder characterized by a diffuse palmoplanter hyperkeratosis and rapidly progressive and devastating periodontitis, affecting the primary as well as the permanent dentition, attributed to a point mutation of the cathepsin C gene. This paper presents a clinical presentation and a brief review of its etiology and treatment modalities.