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Malignant rhabdoid tumor of kidney and brain in an infant. BACKGROUND: Malignant rhabdoid tumors of kidney are associated with atypical teratoid rhabdoid tumors of brain, both being characterized genetically by deletion/ mutation of SMAR CBI/ INI gene. CASE CHARACTERISTICS: 6-month-old male presented with a brain tumor and was subsequently found to have malignant rhabdoid tumor of kidney. INTERVENTIONS: Surgical resection of brain tumor followed by chemotherapy and subsequently resection of renal tumor. OUTCOME: Child died seven months after initial presentation. MESSAGE: Children presenting with embroynal brain tumor, should be investigated for renal tumors and vice versa. MESSAGE: Children presenting with embroynal brain tumor, should be investigated for renal tumors and vice versa.