Pyoderma gangrenosum is a rare ulcerative cutaneous disorder commonly associated with systemic diseases such as inflammatory bowel diseases. This paper reports a case of pyoderma gangrenosum affecting the mouth and the skin associated with ulcerative colitis. A Medline search in English literature from 1961 to 2012 revealed only 11 documented cases of pyoderma gangrenosum with oral involvement. Previous reported cases of oral cavity involvement have shown that the most common sites of manifestation are the tongue, palate, and tonsillar fauces. This case emphasizes the importance of including inflammatory bowel diseases and their manifestations in the differential diagnosis of ulcerative lesions in the oral cavity.