Trabectedin is indicated for patients with advanced soft tissue sarcoma after failure of treatment with anthracyclines and ifosfamide or for patients who are unsuited to receive these agents. The agent has shown activity in patients with advanced uterine leiomyosarcoma, with an acceptable safety profile. Thus, the results of phase II studies have shown that treatment with trabectedin results in 30% progression-free survival at 6 months. More than 50% of these pretreated patients were alive at 1 year. The response rate, progression-free survival, and overall survival compared favorably with other single agents (eg, doxorubicin, ifosfamide, and gemcitabine), with clinical benefit in 50% of patients in second-line treatment. These results are being confirmed in a current prospective phase II study in first-line uterine leiomyosarcoma combining trabectedin with doxorubicin.