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Malignant rhabdoid tumour of kidney is a rare highly aggressive neoplasm of childhood. We present the case of a 18- months old girl presenting with decreased appetite, abdominal distention of 20 days duration and 3 episodes of haematuria. The patient underwent left radical nephrectomy and histopathological examination of the excised specimen confirmed the diagnosis of malignant rhabdoid tumour of the kidney. This case highlights the need to consider malignant rhabdoid tumour of the kidney of possibility young children in presenting with a renal mass.
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