Abstract
•Endometrial mesonephric-like adenocarcinoma (MLA) is a rare and aggressive subtype of epithelial endometrial cancer.•KRAS mutations are characteristic in patients with endometrial MLA.•We report two cases of KRAS-mutated endometrial MLA with excellent and durable responses to lenvatinib and pembrolizumab.•Tumors with KRAS mutations may be particularly sensitive to lenvatinib, but this warrants further study.