Purpose Musicians' practice and performance routines reportedly lead to musculoskeletal complaints and disorders (MCD) that impact their wellbeing and performance abilities. This systematic review aims to assess the prevalence, risk factors, prevention and effectiveness of treatments for MCD in professional musicians and consider the methodological quality of the included studies. Methods A systematic literature search was performed in December 2017 using electronic databases and supplemented by a hand search. Case-control studies, cohort studies, cross-sectional studies, interventional studies and case reports investigating the prevalence, risk factors, prevention or treatment effects of MCD in professional musicians or music students (age ≥ 16 years) were included. Quality assessments of the included studies were performed using an adapted version of the "Study Quality Assessment Tools" from the National Heart, Lung, and Blood Institute. Results One case-control study, 6 cohort studies, 62 cross-sectional studies, 12 interventional studies and 28 case reports were included and assessed for methodological quality. The study designs, terminology, and outcomes were heterogeneous, as the analyses mostly did not control for major confounders, and the definition of exposure was often vague. Therefore, evidence that being a professional musician is a risk factor for MCD as well as the causal relationship between these factors remains low despite the fact that a large number of studies have been performed. Conclusions Studies with high internal and external validity regarding the prevalence, risk factors and effectiveness of the prevention or treatment of MCD in professional musicians are still missing. Further high-quality observational and interventional studies are required.