BACKGROUND Behçet's syndrome is an immune-mediated connective tissue disorder, and its primary manifestations are oral and genital ulcerations. To our knowledge no cases of adverse fetal outcome have been reported in pregnancies complicated by this disease. METHODS A 27-year-old primigravid woman with a diagnosis of Behçet's disease came to our institution during the first trimester. Her pregnancy was complicated by several exacerbations of her disease including vaginal and oral ulcerations and abdominal pain. She was treated with steroids throughout her pregnancy. She had ruptured membranes and evidence of fetal distress at 361/2 weeks and subsequently delivered a severely growth-restricted fetus (< 3rd percentile). CONCLUSIONS Pregnancies complicated by Behçet's disease should be monitored closely for evidence of intrauterine growth restriction and fetal compromise, as are pregnancies complicated by similar connective tissue disorders.