The authors report 2 cases of renal multilocular cyst and review 250 cases reported in the literature, revealing the original features of this benign tumour which has an equal incidence in children and adults with a controversial acquired or malformative aetiopathogenesis, precise histological features, but a difficult preoperative and intraoperative diagnosis despite modern imaging techniques. In the great majority of cases, treatment consists of nephrectomy.

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