Wilms′ tumors is a renal malignancy commonly occurring in childhood with classic histopathological features. While the majority of the tumors present as renal masses, atypical presentations like extrarenal masses have also been known. However, the exact natural course of the disease and its outcome is not known. Here, we present two cases of extrarenal Wilms′ tumor, discuss the pathophysiology and review the literature for the prognostic factors and the treatment outcome.