常染色体隐性多囊肾病是一种以肾集合管和肝内胆管扩张、畸形及肝脏和肾脏纤维化为特点的遗传性疾病。高血压、肾功能不全及门静脉高压是常见的临床症状。影像学表现为肾脏增大但保持肾的外形 ,超声显示肾实质呈弥漫性回声 ,静脉肾盂造影及增强CT显示肾脏呈纹状改变。肝脏影像学表现可正常或肝内胆管扩张 ,门静脉高压常表现脾脏增大和门静脉曲张 Autosomal recessive polycystic kidney disease is a hereditary disease characterized by renal aggregate and intrahepatic bile duct dilatation, deformity and liver and kidney fibrosis. Hypertension, renal insufficiency, and portal hypertension are common clinical symptoms. Imaging findings showed that the kidneys increased but maintained the shape of the kidneys. Sonography showed diffuse echogenicity of the renal parenchyma. Intravenous pyelography and contrast-enhanced CT showed changes in the kidneys. Liver imaging performance can be normal or intrahepatic bile duct dilatation, portal hypertension often showed splenomegaly and portal varices