Aim To investigate the effects of coronary artery stenosis on cardiac pathology and myocardial metabolomics in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Methods Coronary and ventricular sites (anterior, lateral and posterior walls of left ventricle and anterior, posterior wall of right ventricle and interventricular septum) were studied in 35 patients with ARVC undergoing heart transplantation. Coronary artery tissue sections were stained with HE and the degree of stenosis was quantitatively analyzed; slices of ventricular tissue were stained with Masson staining, and the proportion of myocadium, fiber, and adipose tissue in the heart was quantitatively analyzed after image processing. Metabolite extraction and metabolomics analysis of cardiac tissue from coronary artery supply areas were performed. Comparative analysis was conducted on the differences in myocardium, fiber and adipose tissue, and metabolomic profiles among the group of patients without coronary artery stenosis, with mild coronary artery stenosis (＜50%) and with moderate-severe coronary artery stenosis (≥50%). Results Among the 35 patients with ARVC, 10 (28.6%) had moderate-severe coronary artery stenosis, 11 (31.4%) had mild coronary artery stenosis, and 14 (40.0%) had no coronary artery stenosis. The age of patients with moderate-severe coronary artery stenosis receiving heart transplantation was significantly higher than those with mild coronary artery stenosis and those without coronary artery stenosis [(48.5±10.7) years vs. (33.8±10.5) years and (31.0±13.4) years, P＝0.015]. There was no statistically significant difference in the proportion of myocardium, fiber and adipose tissue between patients with moderate-severe, mild coronary artery stenosis and those without coronary artery stenosis in the left ventricular anterior wall, lateral wall, posterior wall, and right ventricular anterior wall, posterior wall, and interventricular septum (P＞0.05). There were 105 metabolites were detected from the metabolic profiles, which attributed to pathways of tricarboxylic acid cycle, amino acid metabolism, purine metabolism, pyrimidine metabolism, pentose phosphate metabolism, glycolysis and gluconeogenesis. Metabolomic analysis showed few differences among the three groups. There was no significant separation of the three groups on heat or in principle component analysis. Conclusion There was no difference in the proportion of myocardium, fiber and adipose tissue of the heart and metabolomic profiles among ARVC patients with moderate-severe, mild coronary artery stenosis and without coronary artery stenosis.