Primary Biliary Cirrhosis (PBC) is a chronic, progressive liver disease associated with markedly elevated serum lipids, but it is not clear if PBC is associated with accelerated atherosclerosis. The present systematic review examined the relationship of PBC to atherosclerotic risk. The lipid abnormalities in PBC are complex, depend on the stage of hepatic dysfunction and affect most lipoprotein classes. Increased cholesterol levels in PBC are primarily due to LP-X, an abnormal LDL particle. LP-X has anti-atherogenic properties and may reduce the atherosclerotic risk. Few studies have examined coronary artery disease (CAD) events in PBC, and none have sufficient sample size of follow-up to determine CAD risk in PBC patients. Nevertheless, one study suggested that 12% of PBC patients died from circulatory system diseases suggesting that lipid treatment is appropriate in some patients. Additional larger scale, prospective studies are required to determine the necessity of lipid treatment in this patient group. In the interim, decisions on the use of lipid lowering agents depend largely on the prognosis of the PBC and physician and patient preference for treatment.