ObjectivesThis systematic review evaluated the safety and efficacy of blood flow restriction exercise (BFRE) on skeletal muscle size, strength, and functional performance in individuals with neurological disorders (ND).MethodsA literature search was performed in PubMed, CINAHL, and Embase. Two researchers independently assessed eligibility and performed data extraction and quality assessments. Eligibility criteria: Study populations with ND, BFRE as intervention modality, outcome measures related to safety or efficacy.ResultsOut of 443 studies identified, 16 were deemed eligible for review. Three studies examined the efficacy and safety of BFRE, one study focused on efficacy results, and 12 studies investigated safety. Disease populations included spinal cord injury (SCI), inclusion body myositis (sIBM), multiple sclerosis (MS), Parkinson's disease (PD), and stroke. A moderate‐to‐high risk of bias was presented in the quality assessment. Five studies reported safety concerns, including acutely elevated pain and rating of perceived exertion levels, severe fatigue, muscle soreness, and cases of autonomic dysreflexia. Two RCTs reported a significant between‐group difference in physical function outcomes, and two RCTs reported neuromuscular adaptations.ConclusionBFRE seems to be a potentially safe and effective training modality in individuals with ND. However, the results should be interpreted cautiously due to limited quality and number of studies, small sample sizes, and a general lack of heterogeneity within and between the examined patient cohorts.