Extra renal Wilm's tumour (EWT) is an exceedingly rare entity and needs to fulfill certain criteria. Of the 48 cases reported, only about 36 have been found to be convincingly proved. We present one more well proven case of EWT and have extensively reviewed the literature. The diagnostic, staging and management guidelines are discussed. Amongst the reported cases, the behaviour of the tumour appears to parallel that of similarly staged "intra renal" Wilm's tumour. Hence the staging and the management protocols of "intra renal" Wilm's tumour can well be applied to tumour of extra renal location albeit with some modifications.