1 | INTRODUCTION
Wilms tumor, also referred to as nephroblastoma, accounts for ~90% of all pediatric renal malignancies[1]. However, extrarenal Wilms tumor (ERWT) manifestation remains rare, occurring in approximately 3% of all Wilms tumor cases[2]. Such extrarenal tumors typically present in the form of an asymptomatic mass within the abdomen, although they can be accompanied by abdominal pain, hematuria, and/or hypertension, with each occurring in roughly a quarter of such cases[3]. Dilated cardiomyopathy and congestive heart failure (CHF) are rare presentations for such tumors, with only a few previously reported cases exhibiting these symptoms. There have not been any published reports to date of a case of ERWT presenting with hypertension, dilated cardiomyopathy, and CHF. Here we present the first case of ERWT with these rare symptoms and review the relevant literature.