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This report presents two cases of Papillon-Lefévre syndrome (PLS) affecting two girls among five siblings belonging to a south Indian Muslim family. The patients were 12 and 14 years old. The patients presented with palmar-plantar hyperkeratosis which started around the age of two years. The elder patient was edentulous due to severe destructive periodontitis causing premature loss of teeth. The younger patient had severe destructive periodontitis with multiple periodontal abscess and loose teeth.