Papillon-Lefevre Syndrome (PLS) is a rare disorder that follows an autosomal recessive pattern of inheritance. It has a prevalence of one to four per million individuals, and carriers are thought to be present in two to four per thousand individuals. PLS is characterized by palmar and plantar keratosis and severe early onset periodontitis affecting both deciduous and permanent dentition. This article focuses on a characteristic presentation of Papillon-Lefevre syndrome.