A 57-year-old female suffered from left flank pain for several weeks. Bilateral renal cysts were diagnosed by ultrasonography of the kidney. Sono-guided aspiration and ethanol sclerotherapy were performed for the left renal cyst. Painless gross hematuria was found 14 days later. Retrograde pyelography revealed that the renal cyst communicated with the left collecting system. Un-roofing of the left renal cysts was performed and multiple simple renal cysts communicating with the left collecting system were also noted. To our knowledge, no such case has been reported in the English literature. We also describe the clinical features and management of this disease.