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We presented a case of tuberous sclerosis (TS) associated with subependymal giant cell astrocytoma (SEGC) and renal angiomyolipoma (RAML). A 19-year-old female, who had been diagnosed as TS since she was 3 months old, was admitted with complaints of headache, vomiting, and abdominal pain. At 10 years of age, a ventricular tumor was shown on CT, and at 16 years of age a ventricular peritoneal shunt was placed for obstructive hydrocephalus. On admission, an abdominal CT showed bilateral renal huge multicystic tumors with hemorrhage, which were diagnosed as RAMLs. CT and MRI showed an intraventricular tumor near the foramen of Monro, and this tumor was removed through a transcortical approach. The pathological diagnosis was SEGA. Large sized RAMLs were identified by CT. Although TS is often associated with additional tumors in the brain, heart, kidney, and other organs, the combination of SEGA and RAML is quite rare. If ventricular peritoneal shunt is placed in a TS patient, risk of shunt malfunction should be taken into account.
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