The authors report a new case of an exceptional benign renal tumour: nephronogenic nephroma, composed of differentiation of the blastema into primitive nephronic formations. It raises the problem of differential diagnosis with adult Wilms' tumour and renal blastematosis. In this patient, it was associated with segmental xanthogranulomatous pyelonephritis, presenting in the form of psoïtis and an abscess of the thigh. Total nephrectomy was performed.
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