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Wilms tumor in children has cure rates of 90% at 5 years with a combined modality of treatment with surgery, chemotherapy and/or radiation. Wilms tumor recurs in 15–20% patients and almost all of them occur within 2 years of diagnosis. Late recurrence in Wilms tumor is rare with only a handful of case reports. No clear cut guidelines exist for management of recurrent Wilms tumor. The outcome with early and late relapses remain similar. Here we report a case of Wilms tumor presenting with recurrence 20 years after first diagnosis and review the literature on very late recurrences.