Introduction:Papillon-Lefèvre syndrome(PLS)is an autosomal recessive disorder characterized by a diffuse palmoplantar hyperkeratosis and early periodontal destruction.Only a few PLS cases are reported by dermatological clinicians due to its rarity.Here,we reported a 16-year-old Chinese boy presented with diffuse transgradient palmoplantar hyperkeratosis since 4 years of age.Case presentation:The patient had also experienced recurrent episodes of swollen gums with premature loss of teeth.He is treated with multidisciplinary approaches.On follow-up,he continued to have recurrent gingival inflammation.Discussion:The etiopathogenesis of PLS is obscure and its management presents a special challenge,which call for in-depth studies that will reveal the complex interactions of genetic,immunologic,and microbiological factors involved in pathogenesis of PLS.Conclusion:PLS is a very rare genodermatosis with characterized palmoplantar hyperkeratosis.Dermatological clinicians should be aware of this rare clinical entity to promote its early diagnosis.