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Symptoms of tuberous scelrosis (TS) are mainly related with brain and kidneys. Seizure, mental retardation, other behavioral problems are dominant. A spectrum of renal tumors from benign angiomyolipoma (AML) to polycystic kidney disease, and rarely malignant renal cell carcinoma have been observed. Cystic AML is a rare phenotype of AML. No case of TS with renal cystic AML has been reported in Korea yet. And chronic kidney disease (CKD) in TS has been seldom reported. We experienced a TS case accompanied by renal cystic AML and CKD diagnosed in a 48-year-old female patient who was hospitalized for left side weakness and seizure under the diagnosis of acute cerebral infarction.