In Malaysia, incidence of Wilms’ Tumor (WT) is rare, with only 8 cases reported over four years according to the Malaysia Cancer Registry Report (2012-2016). Reports of spontaneous rupture of WT are scarce and the commonest presentation of this entity is a palpable mass in the abdomen. Herein, we report on the unusual presentation of an 11-year-old boy with typical symptoms of acute appendicitis. The child was subjected to an initial open appendicectomy and diagnosed intra-operatively with a right sided ruptured renal mass. Subsequent investigation leading to the diagnosis of a ruptured Wilms’ tumor and its treatment is discussed in this case presentation.