Mulibrey-Nanism (Muscle-liver-brain-eye nanism= dwarfism) is a rare syndrome. The underlying TRIM37 mutation predisposes these children to develop tumors frequently. Wilms tumor has been reported to be associated with this syndrome. Here we report a 2-year-old boy with Wilms tumor and Mulibrey-Nanism and review the published similar cases. Our patient had associated atrial septal defect, atrial flutter and an episode of cardiac arrest along with other features of Mulibrey Nanism. We managed him successfully with chemotherapy and surgery. A total of 14 cases (including present case) of Wilms tumor have been reported in Mulibrey-Nanism. Longest survivor has been 15-year-old.