Wilms tumor is extremely rare in adults. There is no standard treatment for Wilms tumor in adults, and the therapy protocols are based on those used in children. Here, we report a case of Wilms tumor in a 24-year-old woman who was effectively treated with apatinib, a small-molecule inhibitor of vascular endothelial growth factor receptor-2. The favorable outcome suggested that antiangiogenic therapy might be effective in treating adults with Wilms tumors. Starting with this case, we reviewed the features of Wilms tumors in adults and its treatment with antiangiogenic therapy. The information in this review could provide a novel approach to treating adults with Wilms tumors.