A 9-year-old girl was treated for the periodontal component of Papillon-Lefèvre syndrome, an autosomal recessive disease characterized by palmarplantar hyperkeratosis and premature loss of teeth. Initially, the patient was found to have a polymorphonuclear leukocyte chemotactic dysfunction, defective leukocyte adherence, and deep periodontal pockets harboring presumptive periodontopathic bacteria. After unsuccessful treatment with combined mechanical therapy and 2 different antibiotics, all of the patient's erupted teeth were extracted in an attempt to minimize the chance of infection of teeth yet to erupt. At age 16 years, the now-erupted teeth have normal gingiva and crevice depths, radiographs show no evidence of periodontal pathology, no periodontopathic bacteria are detected in gingival crevices, and leukocyte function is normal.