A deterioration of liver function may occur during pregnancy in patients with chronic liver disorder. Primary sclerosing cholangitis (PSC) is a chronic progressive liver disorder with a highly variable and fluctuating course. This study aims at investigating the outcome of pregnancy in patients with PSC and, conversely, the effect of pregnancy on the disease. Thirteen pregnancies in 10 patients with PSC (4 with liver cirrhosis, 6 with mild liver disease) were observed. Seven patients had PSC before pregnancy, 2 developed the disease during pregnancy, and one patient developed PSC 2 months after a normal pregnancy with a normal delivery. Clinical symptoms and biochemical analyses were routinely evaluated during the pregnancy. No gastrointestinal haemorrhage was observed during the pregnancy. Two patients had pruritus and 2 abdominal pain before pregnancy, and these symptoms continued during pregnancy. Abdominal pain was noted in 3 patients lacking this symptom before pregnancy. Four patients without pruritus prior to pregnancy developed this symptom during the pregnancy. In two patients, pruritus was so intense as to bring on premature delivery. Liver tests did not indicate any deterioration during pregnancy. No fetal loss occurred. The outcome for all babies was normal. In patients with PSC pregnancy does not seem to have a negative effect on the disease process, neither mothers nor babies showed any ill effects. PSC has not worsened during the pregnancy in our patients.