Pigmented villonodular synovitis (PVNS) is a rare disorder occurring in synovium, bursa or tendon sheath. It is highly proliferative and usually involves the knee and hip joints, it can also occur in ankle and other sites. It has two types, i.e., localized or diffuse. Although diffuse PVNS is rarely lethal and rarely metastases, it is difficult to completely resect and has a high recurrence rate. Evidences now suggest a neoplastic origin for PVNS, including CSF-1 gene overexpression. With this discovery, targeted medical therapies have been developed. As novel methods of treatment for pigmented villonodular synovitis, some of them have promising results. More researches are required to understand the definite efficacy of such new targeted agents. In this article, we reviewed current and future treatments of pigmented villonodular synovitis.