A low-carbohydrate, high-fat (LCHF) diet has been proposed to enhance the fat utilization of muscle and the aerobic capacity of endurance athletes, thereby improving their exercise performance. However, it remains uncertain how the macronutrient intake shift from carbohydrate to fat affects endurance exercise training and performance. This study performed a systematic review and meta-analysis to explore the effects of a ketogenic low-carbohydrate, high-fat (K-LCHF) diet on aerobic capacity and exercise performance among endurance athletes. Searches were carried out in five electronic databases, and we followed the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines. The search included studies using an LCHF diet as an intervention protocol and compared data on factors such as maximum oxygen uptake (VOmax) and rating of perceived exertion (RPE) from the graded exercise test. In this case, 10 studies met the criteria and were included in the meta-analysis. We did not find a significant effect of K-LCHF diet interventions on VOmax, time to exhaustion, HRmax or RPE. However, a significant overall effect in the substrate oxidation response to respiratory exchange rate was observed. The meta-analysis showed that K-LCHF diets did not affect aerobic capacity and exercise performance. Therefore, high-quality interventions of a K-LCHF diet are needed to illustrate its effect on various endurance training programs.