Both qualitative and quantitative neutrophil abnormalities have been associated with severe forms of periodontitis. Defects in chemotaxis, phagocytosis and bacterial killing have been reported among both peripheral blood and gingival neutrophils harvested from patients with juvenile and rapidly progressive periodontitis. Chronic granulomatous disease of childhood (CGD) is a rare, inherited disorder associated with the occurrence of severe, life-threatening, suppurative infections of skin, liver, lymph nodes and other organs. Neutrophils and monocytes from individuals with CGD lack enzymes necessary for the production of oxygen reduction/products such as H2O2 and superoxide anion, and therefore are unable to kill many species of bacteria and fungi. However, no detailed study of the periodontium of these patients has been undertaken. Accordingly, five patients whose ages ranged from 17 to 32 years were included in this study. An additional (sixth) patient was included based on complete dental records. Neutrophils from all patients demonstrated defective O2 metabolism, and all patients had histories of chronic recurrent abscesses consistent with CGD. All patients were receiving antibiotic prophylaxis. Several patients had ulcerative lesions of the oral cavity of unknown etiology. Examination of the periodontium revealed that three patients had gingivitis, one had localized early periodontitis, and one had generalized early-to-moderate periodontitis. The severity of periodontal disease was consistent with patient age and local etiologic factors. No patients had evidence of juvenile, severe or rapidly-progressing disease in spite of their leukocyte defects. These findings suggest the following possibilities.(ABSTRACT TRUNCATED AT 250 WORDS)