Nephroblastomatosis is a paediatric renal disease that may undergo malignant transformation. When neoadjuvant chemotherapy is indicated for nephroblastomatosis or bilateral Wilms' tumours, exact volumetric analysis using high-speed data processing and visualization may aid in determining tumour response. Using 3D-volume-rendering software, the 0.5-T MRI data of a 2-year-old girl with bilateral nephroblastomatosis was analysed. Exact volume determination of foci of nephroblastomatosis was performed by automatic and manual segmentation, and the relation to normal renal parenchyma was determined over a 12-month period. At the first visit, 80% (460/547 ml) of the extremely enlarged right kidney was due to nephroblastomatosis. Total tumour volume within the right kidney decreased to 74 ml under chemotherapy. Volume analysis of the two emerging right-sided masses after treatment correctly suggested Wilms' tumour. Three-dimensional rendering of the growing masses aided the surgeon in nephron-sparing surgery during tumour resection.