Wilms tumors are the most common renal malignancy of childhood, often presenting as asymptomatic abdominal masses. These tumors can occur either in a unilateral or bilateral fashion, with bilateral tumors more commonly associated with various genetic syndromes and familial inheritance with some known loci. Bilateral tumors present challenges with regards to balancing oncologic control with renal function. Here, we present an unusual case of a young female patient diagnosed with bilateral Wilms tumors on the background of a strong family history and unknown genetic loci.
