Intrahepatic cholestasis of pregnancy (ICP) is a rare disorder of unknown etiology with a symptomatically distressing maternal course with pruritus as the chief complaint. ICP poses little medical risk to the mother, but poses significant risk to the fetus of perinatal mortality, preterm delivery, fetal distress, and meconium staining. ICP has a geographically variable prevalence and appears to have a heritable component. Current evidence suggests a susceptibility to derangements in the sulfation of steroid compounds, affecting the metabolism of progesterone and bile acids in the fetal/placental compartment. This impairs transport of bile acids across the placenta from the fetal to the maternal circulation. Exactly how this leads to fetal compromise is unknown. The most efficacious current medical management that improves both maternal symptoms and laboratory abnormalities is ursodeoxycholic acid (UDCA), a hydrophilic bile acid that alters the composition of the bile acid pool in maternal blood. When ICP is diagnosed, UDCA coupled with close maternal-fetal surveillance is indicated. Delivery should be effected near term, with confirmation of fetal lung maturity or earlier if fetal compromise is identified.