Papillon-Lefevre syndrome is an extremely rare autosomal recessive genetic disorder characterized by hyperkeratosis of palms soles and premature loss of both deciduous and permanent teeth. Recent theories suggest that this condition is linked to a mutation of the Cathepsin-C gene. Here present two cases of the Papillon-Lefevre syndrome from two distinct families, both of which exhibit all the recognizable symptoms. Both patients presented with persistent thickening of the skin of palms and soles. Severe generalized periodontal destruction and aggressive periodontitis with mobility of teeth showed as intraoral features and orthopantomography showed the generalized alveolar and arc-shaped bone loss in both patients.

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