The main therapeutic strategy for metastatic Myxofibrosarcoma (MFS) is palliative chemotherapy. A number of studies have demonstrated that anti-angiogenic therapy and immunotherapy could improve the survival rate of patients with metastases. However, the effectiveness of the combination of anti-angiogenic therapy and immunotherapy for the therapy of MFS is undetermined. The current study reports a case of metastatic myxofibrosarcoma that was treated with combination Nivolumab (monoclonal antibody, PD-1 inhibitor) and Bevacizumab (monoclonal antibody, anti-VEGF) after progression from the single use of Nivolumab. The aim of the current study is to assess the efficacy and safety of Nivolumab and Bevacizumab for metastatic myxofibrosarcoma and to review the literature. Up to the termination of the follow-up, the patient achieved a partial response for 16 months, had an overall survival for over 29 months since the metastasis and demonstrated a sustained benefit from treatment. The most frequent adverse events were fatigue, abnormality of Alanine aminotransferase (ALT), hypertension and proteinuria. Nivolumab and Bevacizumab treatment indicate beneficial clinical effects and are indicated to be safe to use in patients with metastatic myxofibrosarcoma.