The periodontal status of patients with laryngo-onycho-cutaneous syndrome is unknown. This study describes a case of severe aggressive periodontitis in a 19-year-old American-Pakistani female with laryngo-onycho-cutaneous syndrome. The patient showed abundant dental plaque and calculus accumulations, suppuration and hemorrhage from virtually all gingivae and interdental papillae, and extensive radiographic alveolar bone loss and vertical mobility of several teeth. Subgingival plaque samples revealed a variety of major periodontal pathogens. The patient was scheduled for full-mouth tooth extraction.