Pyostomatitis vegetans is a rare chronic disorder of the oral mucosa. It is a highly specific marker for inflammatory bowel disease. Although immunological and microbial factors have been suggested as predisposing factors, the exact aetiology is unknown. Here, a case of pyostomatitis vegetans in a 30-year-old woman is reported. No bowel involvement was observed. Report of such cases emphasizes the importance of early diagnosis of oral findings in control and management of disease.