Adenosarcomas are the rarest form of uterine sarcomas, and clinical experience with their management is still limited. Here, we reported 7 patients with uterine adenosarcoma referred to our institution, focusing on main pathologic features, their medical history, and long-term follow-up. Among these patients, we provided a detailed description of the medical history of a 49-year-old woman with advanced uterine adenosarcoma with sarcomatous overgrowth who presented a brilliant radiologic and pathologic response after 3 cycles of epirubicin and ifosfamide, ultimately achieving an extraordinary long-term outcome through an integrated surgical and medical approach. Our single-centre experience would suggest that aggressive uterine adenosarcomas with sarcomatous overgrowth are sensitive to standard epirubicin and ifosfamide and that an integrated approach, both medical and surgical, could be considered in clinical practice, again emphasizing the relevant role of multidisciplinary management for this extremely rare disease.