Background: Evidence for the long-term health effect of low-carbohydrate diets (LCDs) is inconsistent. Herein, we aimed to examine the associations of LCDs with cardiovascular disease (CVD) and all-cause and cause-specific mortality. Methods: We searched PubMed, EMBASE, and the Web of Science up to 26 July 2023 for eligible publications. Random-effect models were used to pool the summary relative risks (RRs) and 95% confidence intervals (CIs). Results: A total of 44 studies (17 articles) were included in the systematic review and 38 in the meta-analysis, including 223 657 all-cause deaths (771 609 participants), 14 046 cardiovascular deaths (274 807 participants), 18 264 CVD cases (405 631 participants), and 3634 coronary heart disease (CHD) cases (151 023 participants). Subsequently, the highest LCD score was compared with the lowest one and the pooled RRs (95% CIs) were 1.05 (0.96, 1.14; I2 = 65.1%; n = 13) for CVD, 1.43 (1.18, 1.72; I2 = 25.4%; n = 3) for CHD, 0.93 (0.81, 1.06; I2 = 0.0%; n = 2) for stroke, 1.03 (0.96, 1.10; I2 = 86.6%; n = 13) for all-cause mortality and 1.09 (0.99, 1.19; I2 = 65.1%; n = 10) for cardiovascular mortality. Conclusion: Our analysis showed positive associations of LCDs with CHD. Thus, vigilance is recommended for long-term adherence to this dietary pattern.