Introduction and Objective: Esophageal squamous cell carcinoma (ESCC) is the eighth most common cause of cancer-related death worldwide. However, previous genome-wide single nucleotide polymorphism association analyses have not explained the high heritability associated with ESCC. The aim of this study was to investigate if genes copy number variations (CNVs) are associated with the presence of ESCC. Data sources: We performed a search of MEDLINE and EMBASE databases on the 21st August 2023. Study selection: Case-control studies assessing the association of at least one copy number variation with ESCC were included. Data extraction: Data were independently extracted by two reviewers. A random effects model was used to calculate combined odds ratios for commonly investigated CNVs. Results: 15 studies examining genes CNVs were identified. 3 CNVs were assessed in at least 3 studies and included in a meta-analysis. Results showed an association of ABCC4, erbB2 and MCL1 CNVs with ESCC presence. Conclusion: ABCC4, erbB2 and MCL1 genes might therefore have predictive and therapeutic potential for ESCC.