Papillon-Lefevre syndrome (PLS) is an autosomal recessive disorder that begins in early childhood and is extremely rare. It is characterized by aggressive periodontal disease, palmar plantar hyperkeratosis, a tendency for dry and chapped skin, and thin sparse hair. Patients may exhibit early tooth loss between the ages of 2 and 4, followed by premature loss of permanent teeth during adolescence. The etiopathogenic factors that are associated with PLS includes genetic, immunological, and microbial factors. A 4-year-old boy presented with a chief complaint of multiple losses of teeth and an unsatisfactory aesthetic appearance. His gingiva appeared edematous, bright red, and bled readily upon probing, and the teeth were mobile. Hyperkeratosis was observed on both the palms and soles. These findings are consistent with Papillon-Lefèvre syndrome. Early detection and diagnosis of Papillon-Lefevre syndrome play important roles in improving the physical and mental well-being of the patients.