Objective To investigate the CT and MR imaging features of adult congenital polycystic kidney and comparison with pathology, and to provide a theoretical basis for the diagnosis of adult congenital polycystic kidney. Methods The clinical manifestations of 100 patients with adult congenital polycystic kidney treated in our hospital from Jan 2104 to Jan 2017 were abdominal mass and hematuria. All patients received CT and MRI examinations, the pathological sections were subjected to hematoxylin and eosin (HE) staining and pathologically observed and compared with the results of CT and MRI. Results All patients suffered the renal parenchymal involvement, the mean size of renal cyst was (43.34±6.45) mm, normal renal parenchyma was replaced by different volumes of cysts with a smooth edge and a uniform density, the mean CT value was (56.23±4.32) Hu, pathological examination showed small stones near the capsule wall, cyst with calcification was curved or lamellar, the surrounding organs were displaced. Pathological examination showed that the cyst wall was mainly composed of fibrous tissue, and tubular epithelium was observed, normal structure of kidney tissue was not founded. Conclusion The CT and MRI imaging features of polycystic kidney correspond to their pathological components.