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Giant Hydronephrosis in the adult can reach elephantoid proportions, containing several liters of fluid. It can be associated with rare renal neoplasms. We present a 27 year old man with progressive painless abdominal distention whose imaging investigations (ultrasound and CT scan) disclosed a left GH. This was successfully removed en-bloc by simple nephrectomy via a midline laparotomy. Obstruction was at the UPJ, the mass contained 13.5L of fluid and histopathologic examination revealed Angiomyolipoma of the kidney and pelvis. This is
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