Intrahepatic cholestasis of pregnancy is characterized by pruritus, raised maternal liver enzymes and bile acids. It usually presents in the third trimester and rarely before 25 weeks. Ursodeoxycholic acid is the most promising therapy to alleviate symptoms and prevent fetal risk. However, its administration has been restricted for the last trimester as its embryotoxic effect is undetermined. We report here a case of an extremely rare, severe obstetric cholestasis with early onset treated by ursodeoxycholic acid from the 9th week of pregnancy, the earliest ever reported in the literature. The treatment was well tolerated. At the 32nd week urgent caesarean section was performed due to intolerable symptoms, worsening laboratory results and signs of fetal distress. A healthy newborn was delivered. It is concluded that ursodeoxycholic acid in case of a severe early-onset intrahepatic cholestasis may be started in the early pregnancy to improve maternal condition and prevent fetal complications.