Turner syndrome is a chromosomal disorder in which of all or part one of the X chromosomes is absent, and this syndrome affects females. The syndrome leads to a constellation of physical findings that often includes congenital lymphedema, a short stature and gonadal dysgenesis. The prevalence of inflammatory bowel diseases in the general population is estimated to be 150∼250 per 100,000 population. A 2-fold increase in the risk of developing inflammatory bowel diseases has been reported in women with Turner syndrome. We describe here a case of Turner syndrome with Crohn's disease. (Korean J Gastrointest Endosc 2010; 40:275-279)