Uterine leiomyosarcoma (U-LMS) is the most common histotype among all primary uterine sarcomas. The lung, peritoneum, pelvic or para-aortic lymph nodes, bone and liver are the most common metastatic sites involved. However, some case series of atypical sites of metastasis such as heart, brain and different endocrine glands have been described. Here we report the clinical presentation and therapeutic management of two patients affected by U-LMS with a very atypical behavior, characterized by soft tissue metastases with an impressive and massive enlargement during chemotherapy despite a good control of the other sites of disease.