A very critical feature in women's health is the identification of risk factors for pregnancy and adverse fetal outcome. Primary biliary cirrhosis is an autoimmune disease of the liver that predominantly affects older women. However, the serologic onset of this disease appears to precede clinical manifestations by many years. The goal of this case controlled study was to analyze fertility in primary biliary cirrhosis (PBC) and investigate the outcome of pregnancy, and the influence of pregnancy on the course of the disease. The study included 233 consecutive female patients with PBC seen between 1987 and 2012. Among them, 186 had at least one conception and were matched for age with a 1:2 group of controls (367 healthy women with at least one conception in their life). PBC patients experienced 507 pregnancies as opposed to 700 pregnancies among controls (mean 1.91 vs 2.73, p < 0.05). The two groups' life history was similar in terms of miscarriages, voluntary interruptions of pregnancy, and term and preterm deliveries. The rates for one or more cesarean deliveries were lower for PBC patients (5.7 vs 11.7 %, p < 0.05). Pruritus during pregnancy was recorded in 15 pregnancies involving 13 PBC patients (3.0 %) and none of the controls. Perinatal and postnatal deaths and complications at childbirth were only recorded in the PBC patients, involving a total of 11 babies (2.7 %, p < 0.05). Eight pregnancies occurred after PBC was diagnosed in six patients, all of which had a favorable course at term, with no complications at childbirth. Ursodeoxycholic acid was continued during pregnancy and no exacerbation of the disease was observed. In conclusion, successful completion of pregnancy is a realistic expectation for PBC patients, though pregnancy and delivery must be monitored for the potentially higher than normal risk of childbirth complications.