Fibropolycystic liver diseases (FLDs) is a rare genetic disorder, including bile duct hamartomas, congenital hepatic fibrosis, polycystic liver disease, Caroli’s disease, and choledochal cysts. Fibropolycystic liver diseases has received little clinical attention and exhibits a variety of imaging manifestations, leading to a high likelihood of missed diagnosis and misdiagnosis. Through this case, we delineate the characteristic imaging manifestations of the disease and its underlying pathological mechanisms. Our objective is to enhance readers' comprehension of the disease and thereby reduce the rate of missed diagnosis and misdiagnosis of the disease.