Papillon-Lefèvre syndrome (PLS) is a rare genetic disorder with autosomal recessive inheritance, typically manifesting in childhood. The hallmark features include palmoplantar hyperkeratosis and premature loss of teeth. Additional associated findings may include psoriasiform plaques on the knees and elbows, hyperhidrosis, recurrent pyogenic infections, intracranial calcifications, and both physical and mental developmental delays. This case report presents a 5-year-old child who was admitted to the Faculty of Dentistry at Kırıkkale University with tooth loss and was subsequently diagnosed with Papillon-Lefèvre Syndrome, accompanied by a review of the relevant literature.