4 year old child, presented with c/o mass in the right side of the abdomen noticed for the last 15 days which was gradually increasing in size. The child was evaluated and ultrasound and CECT abdomen was suggestive of large cystic right kidney mass of approx 10X8 cm, localized to the upper pole of the right kidney. The lower pole of the right kidney was spared. In view of the localized upper pole cystic tumor and imaging suggestive of benign lesion, the child was planned for renal sparing surgery along with frozen section biopsy. The child underwent excision of the tumor salvaging the lower part of the right kidney, the frozen section was suggestive of benign lesion. The biopsy confirmed the diagnosis of cystic nephroma, which is a very rare benign kidney tumour in children.
The commonest renal tumor in the paediatric age group is Wilm’s tumor which is a malignant tumor, which requires nephrectomy along with chemotherapy. While cystic nephroma is generally considered as a benign end of the spectrum. In these types of tumor renal sparing surgery should be considered whenever feasible.