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To determine the functional outcomes, complications and revision rates following total knee arthroplasty (TKA) in patients with pigmented villonodular synovitis (PVNS).We conducted a systematic review of the literature. Five studies with a total of 552 TKAs were included for analysis. The methodological quality of the articles was evaluated using the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) scale. Functional outcomes, complications and revision rates were assessed. The mean age was 61 years (range 33–94 years) and the mean follow-up period was 61.1 months (range 0.2–35 years). All the studies reported improvement in knee function following TKA. Post-operative stiffness was the most frequently reported complication, affecting 32.7% ( n = 32) of patients in our review. Symptomatic recurrence of PVNS, component loosening, tibial-component fracture, instability and periprosthetic infection were the main factors leading to the need for revision TKA. The findings of this review support the use of TKA to alleviate the functional limitations and pain due to knee degeneration in patients with PVNS. The operating surgeon should be aware of the increased risk of post-operative stiffness, as well as a potentially higher risk of infection. Implant survival should also be considered inferior to the one expected for the general population undergoing TKA.