At present, the treatment strategies in patients with localized and diffuse forms of pigmented villonodular synovitis have more or less been standardized. However, these strategies are not optimal because high recurrence rates persist and studies with a sufficient level of evidence are lacking. This systematic review article describes all known treatment options for intra-articular pigmented villonodular synovitis and their clinical results. Based on this research, we provide guidelines to support physicians in making the optimal treatment decisions. Given the rarity of the disease, randomized studies are not to be expected, but an international registry through existing networks would offer the benefit of getting a better insight into the outcome of this disease. Therefore, we propose a basic set of data to be investigated and ideally to be reported on in such a registry.