Objective To summarize the imaging findings of children with multicystic dysplastic kidney and evaluate the diagnostic value of CT. Methods The clinical data of 16 children with clinical and imaging confirmed MCDK treated in our hospital between January 2010 and July 2016 were analyzed retrospectively. The examination methods and imaging findings were summarized. Results CT plain scan showed that there was grape cluster-like low density shadow in kidney, composed of unequal size and different number of cysts and there was not communicated separation between cysts. Enhanced scan showed that there was no enhancement of cyst, moderate enhancement of separation. Delayed scan showed that the contrast agent did not enter the capsule. MRI scanning showed that in kidney, there were many low signals on T1WI, high signal cystic shadow on T2WI. The cystic wall and separation were clearly displayed. There were equal signals on both cyst T1WI and T2WI. MRU showed that the kidney of patient displayed a number of different sizes of isolated cystic high signal shadow, and there was no exchange between capsules. Intravenous renal pelvis radiography examination showed that the kidneys were not developed. Conclusion CT and MRI of MCDK have obvious imaging characteristics. CT enhanced scanning is good for displaying poorly developed small kidneys, cystic wall and separation and other parenchyma parts. The disease can be basically diagnosed.