Pyostomatitis vegetans (PV) is a rare inflammatory stomatitis often associated with inflammatory bowel diseases (IBD). Treatment of PV depends on the presence of coexisting IBD. To date, there have been no reports on the use of ustekinumab for PV. There have been recent cases on the use of ustekinumab for treatment of pyoderma gangrenosum and uveitis. We report the case of a 26-year-old female with moderately severe Crohn’s disease (CD) and PV who was successfully treated with ustekinumab monotherapy. Since initiation of ustekinumab, she has been in clinical and biochemical remission from a CD and PV standpoint for the last 12 months.