Coronary artery disease (CAD) is a major cause of heart failure with preserved ejection fraction (HFpEF). In studies of HFpEF, the reported prevalence of CAD varies widely, which may be the result of inconsistent definitions of CAD, geographic and ethnic differences in CAD burden, varying definitions of HFpEF (including different cutoffs for "preserved ejection fraction"), and differences in study design. Despite these limitations, pooled analysis of prospective HFpEF studies demonstrates that CAD is common in HFpEF, with an estimated prevalence of approximately 50%. Based on available data, patients with signs and symptoms of heart failure who have preserved left ventricular ejection fraction and evidence of CAD (HFpEF-CAD) most likely comprise a distinct etiologic and pathophysiologic subset of HFpEF. Therefore, future clinical trials in HFpEF should a priori stratify by CAD or specifically target patients with CAD, strategies that may improve the disappointing track record of therapies tested in HFpEF. The combination of systematic evaluation and management of CAD in HFpEF, along with promising future therapies for HFpEF-CAD, may lead to improved outcomes for this challenging clinical syndrome.