<p>This study reports a rare case of Caroli disease (CD) combined with pregnancy, and analyses and discusses the etiology, differential diagnosis, clinical treatment of CD, and its special characteristics during pregnancy; this study suggests that medical and surgical comorbidities occurring during pregnancy should be given due attention, and the possibility of congenital intrahepatic bile duct structural abnormality should be identified, with a view to providing more insights into the diagnosis of CD and improving the level of obstetricians and gynaecologists’ diagnosis and treatment of this rare disease, in order to timely diagnose and aggressively treat it, thereby improving maternal and infant outcomes. This study suggests that the possibility of congenital intrahepatic bile duct structural abnormalities should be identified in order to provide more insights into the diagnosis of CD and to improve the diagnosis of this rare disease by obstetricians and gynaecologists, so that timely diagnosis and proactive treatment can be made to improve the outcome of mother and child.</p>