Pyostomatitis vegetans is a rare oral disorder often associated with gastrointestinal and/or cutaneous diseases and sometimes with other disorders. The association of pyostomatitis vegetans with inflammatory bowel disease, particularly ulcerative colitis, is well known and in most cases bowel disease precedes the onset of oral lesions by months or years. A peripheral eosinophilia has been observed in most reported cases. Pyostomatitis vegetans is characterized by erythematous, thickened oral mucosa with multiple pustules and superficial erosions. Histopathologically, it is characterized by intraepithelial and/or subepithelial abscesses containing large numbers of eosinophils. The pathogenesis is as yet unknown although immunological and microbial factors have been suggested as possible aetiological factors. Management of pyostomatitis vegetans consists mainly of immunosuppression using topical or systemic corticosteroids with medical and/or surgical treatment of any underlying inflammatory bowel disease.