Caroli病是一种以非阻塞性肝内胆管扩张为特征的罕见先天性疾病。大多认为是常染色体隐性遗传病，但许多病例无法追寻典型的遗传家族史。Caroli病包括两种类型，简单型（称为Caroli病）和以先天性肝纤维化和/或多囊肾病为特征的Caroli综合征。认为PKHD1基因是Caroli病、Caroli综合征的致病基因。“,”Caroli’s disease is a rare congenital disease characterized by non-obstructive dilatation of the intrahepatic bile ducts, with a prevalence of one in a million in the general population[1]. Most of it is considered to be an autosomal recessive genetic disease, but in many cases, the typical genetic family history cannot be traced back. There are two forms of Caroli’s disease: simple type (commonly called Caroli disease) and Caroli syndrome (characterized by congenital liver fibrosis and/or polycystic kidney disease). PKHD1 gene is considered to be the causative gene of Caroli’s disease, congenital liver fibrosis and/or polycystic kidney disease [2]. Here, we introduce a case of Caroli's disease confirmed by pathology, atypical symptoms and images in our hospital.