"Cardiac cephalgia" is a type of secondary headache disorder, usually initiated by exertion that is related to myocardial ischemia. Primary exertional headaches such as sex-, cough-, or exercise-induced headaches are typically benign. Cardiac cephalgia, on the other hand, can have life-threatening complications. Due to overlapping features and similarities in presentation, cardiac cephalgia can be misdiagnosed as a primary headache disorder such as migraine. However, the management of these conditions is unique, and treatment of cardiac cephalgia with vasoconstrictors intended for migraine can potentially worsen myocardial ischemia. Thus, it is important to make the correct diagnosis by evaluating cardiac function with an electrocardiogram and/or stress testing. In this review, we examine reported cases of cardiac cephalgia from the past 5 years to highlight the importance of this condition in the differential diagnosis of a headache in a patient with a history of cardiovascular risk factors, as well as to discuss the appropriate approach to diagnosis and the proposed pathogenic mechanisms of this condition.