Kussmaul's sign, the absence of a drop in jugular venous pressure or a paradoxical increase in jugular venous pressure on inspiration, can be evaluated as an indicator of right ventricular myocardial infarction. Right ventricular myocardial infarction complicates 30-50% of inferior myocardial infarctions and is associated with increased mortality when compared to inferior myocardial infarction without right ventricular involvement. Early recognition allows maintenance of preload. We reviewed the diagnostic test accuracy studies for Kussmaul's sign for diagnosis of right ventricular myocardial infarction. We conducted a librarian-assisted search using PubMed, Medline, Embase, and the Cochrane database from 1965 to October 2019. Only English language restriction was imposed. We identified studies that assessed patients presenting to a hospital with a suspected myocardial infarction who underwent an assessment for Kussmaul's sign and a diagnostic test for right ventricular myocardial infarction. Four independent reviewers extracted data from relevant studies. Study quality was assessed using the QUADAS-2 tool. A bivariate random effects meta-analysis was performed. We identified 122 studies; ten were selected for full review. Eight studies had comparable populations with a total of 469 consecutive patients admitted with acute inferior myocardial infarction and were included in the analysis. Prevalence of right ventricular myocardial infarction was 36% (confidence interval [CI] 95% 31.8-40.5). All reference standards were combined. Kussmaul's sign had a sensitivity of 62.5% (44.6, 77.5), specificity 90% (73.0, 96.8), negative likelihood ratio (LR) 0.2 (0.1-0.8) and positive LR 5.8 (2.5, 13.3). In the presence of acute myocardial infarction, Kussmaul's sign is specific for acute right ventricular myocardial infarction and may serve as an important clinical sign of right ventricular dysfunction requiring preload preserving management.