Pixantrone is a novel anthracycline derivative, manufactured by Cell Therapeutics Incorporated, WA, USA. It was developed with the aim to retain the efficacy of anthracyclines and be less cardiotoxic. Initial safety trials and single-arm, Phase II trials have shown preliminary evidence of anticancer activity and manageable toxicity. These results were validated in multicenter, randomized clinical trials where pixantrone was used as single agent or in combination with other cytotoxics. Following the results of PIX301, it is now approved by the EMA for use as monotherapy in pretreated patients with refractory non-Hodgkin lymphomas. Ongoing trials are assessing the use of pixantrone in combination with other drugs.