Objective: The aim of this systematic review and meta-analysis was to evaluate the short-term and long-term outcomes of coronary artery bypass grafting (CABG) with coronary endarterectomy (CE) versus isolated CABG. Methods: Studies evaluating outcomes of CABG with CE (CE-CABG) were searched from 1 January 2000 to 30 September 2022, on PubMed, Embase, and Cochrane databases. The primary outcome was 30 -days mortality. Secondary outcomes were postoperative myocardial infraction, low output syndrome, cardiac arrhythmia, renal dysfunction, and 5 years survival. Results: A total of 12 observational studies including 114,319 patients assessing CE-CABG ( n = 35,174) versus isolated CABG ( n = 79,145) were included. Compared to isolated CABG alone, CE-CABG was significantly associated with increased incidences of 30-days mortality (RR, 1.87; 95% CI, 1.73–2.07; p < 0.01), postoperative myocardial infraction (RR, 1.61; 95% CI, 1.26–2.05; p < 0.01), low output syndrome (RR, 1.54; 95% CI, 1.17–2.02; p < 0.01), and renal dysfunction (RR, 1.56; 95% CI, 1.44–1.69; p < 0.01). However, there was no difference in either rate of cardiac arrhythmia (RR, 1.06; 95% CI, 0.97–1.15; p = 0.20) or 5 years survival (RR, 1.05; 95% CI, 0.95–1.16; p = 0.34) between the CE-CABG group and the control group. Subgroup analysis on CE technique showed that CE-CABG was also associated with 30 days mortality in patients undergoing closed CE (RR, 1.49; 95% CI, 1.09–2.03), whereas this association between CE and 30 days mortality was not observed in patients undergoing open CE (RR, 1.76; 95% CI, 0.58–5.32). Conclusions: Despite poor short-term outcomes, CE-CABG appeared to offer satisfactory long-term survival in patients with diffuse coronary artery disease.