The differential diagnosis of jaundice, especially in pregnant women, is often very difficult. The authors use an example of primary sclerosing cholangitis in a healthy female patient during pregnancy to describe the differential diagnosis of diseases accompanied by jaundice. Primary description in the Russian literature, a clinical observation of primary sclerosing cholangitis developed during pregnancy has been presented. It is shown that the occurrence and progression of the disease during pregnancy is extremely unfavorable both for the mother and the fetus. As distinct from the previous foreign studies concerning the analysis of the course of pregnancy in primary or secondary sclerosing cholangitis, authors revealed no positive effect of ursodeoxycholic acid preparations. On the grounds of literature data and own data the authors conclude that primary sclerosing cholangitis, despite rarity of the disease, is necessary to be included in the differential diagnosis of nosological forms with jaundice. At diagnostics of primary sclerosing cholangitis it is necessary to set up in time question about premature delivery or pregnancy termination.