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Background: Castleman Disease (CD) of the kidney is extremely rare. In this study, we have presented a case of CD of the left kidney and comprehensively described the findings of computed tomography urography. Case Presentation: The case involved unusual imaging characteristics of the focal central cystic area. Conclusion: The small and regular cyst-like structures and the hyperdense mass relative to the renal parenchyma in plain scans might help distinguish the CD of the kidney from other hypervascular tumors.