Past medical history included a cholecystectomy, caesarean section and a subcoccygeal neurofibroma excision. Examination showed an incisional hernia over a lower midline scar and a palpable right renal mass.

An urgent CT scan was performed which confirmed that she had a multi-cystic lesion about 10 cm in diameter in the right kidney that contained some solid elements. It also showed that she had some cysts on the ovaries (6.5 cm on the left and 2 cm on the right). Although malignancy could not be entirely excluded, there was no evidence of spread.

A DMSA static renal scan showed that there was only 13% function in the remaining portions of the right kidney. She also continued to have upper quadrant pain and discomfort. A right radical nephrectomy via a right flank approach was performed.

Macroscopic examination (Fig. 1) showed an enlarged kidney of 15 x 13 x 7 cm. Most of it was replaced by a multilocular cystic neoplasm with the cysts having thickened white walls and containing clear serous fluid. Some normal residual kidney was present at one pole and the cystic neoplasm did not communicate with the renal pelvis. Histology of multiple sections (Fig. 2) revealed varying-sized cysts lined by attenuated columnar epithelium with fibrocollagenous walls. No blastema or immature tubules were present.