Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome that is characterized by fibrosing inflammatory destruction of intra- and/or extrahepatic biliary ducts. The etiology is unknown and there is no known effective treatment. The course of PSC is quite variable; however, the disease is typically slowly progressive. There is little known regarding the natural history and potential complications of pregnancy in a patient with PSC. This case report details a 36-year-old woman with PSC who became pregnant. The pregnancy was complicated by the development of dominant stricture. A healthy baby boy was delivered at 33.5 weeks. The mother required cholangiography and stent placement immediately after delivery, but her postpartum course was otherwise unremarkable.