Leukemoid reaction is a rare paraneoplastic syndrome. It can occur in association with carcinomas, in particular of the lung, gastric and renal. However, its association with sarcoma is infrequent. Leukemoid reaction occuring in patients with uterine sarcoma have not been previously reported. We report the case of a « leukemoid » leucocytosis revealing an uterine sarcoma with fatal evolution.