We present a case of Papillon-Lefèvre syndrome in an 8-year-old child. The syndrome is characterized by periodontal disease affecting the deciduous and permanent dentition, along with palmar and plantar keratosis. Other symptoms include increased susceptibility to infections. Family history revealed consanguinity and similar findings in her two siblings. A review of several reports describe successful outcomes in a few patients, which suggests it may be possible to halt the periodontal disease process if one uses a combined and intensive mechanical and antimicrobial treatment approach in these patients.