Intrahepatic cholestasis of pregnancy (ICP) is a poorly recognized and under-diagnosed maternal condition associated with an increased incidence of fetal mortality and morbidity. This article reviews the current understanding of the epidemiology and pathogenesis of the condition, and explores the effects on both mother and fetus. The article concludes with a brief review of possible explanations of the pathophysiology. An understanding of the clinical and laboratory features of this disease will facilitate early diagnosis, which is imperative if the high stillbirth rate in ICP is to be reduced.