Cardiac cephalalgia is a rare manifestation of myocardial ischemia. The diagnostic process is rendered challenging due to its propensity to mimic primary headache disorders and the frequent absence of specific neurological signs. This chapter reviews the pathophysiology, clinical presentation, diagnostic approach, and management of cardiac headache, illustrated by a case report. The pathophysiology involves referred pain via visceral afferent pathways, transient intracranial hypertension due to reduced cardiac output, and inflammatory mediator release during myocardial injury. Clinical presentation typically includes sudden-onset, exertional, intense headache, often diffuse or vertex-localized, potentially accompanied by nausea and symptoms of cardiac ischemia. Diagnosis requires meticulous clinical evaluation, cardiovascular risk assessment, and ancillary testing, including electrocardiogram, stress tests, and coronary angiography. Differentiation from migraine is crucial, as triptans are contraindicated due to potential coronary vasoconstriction, while nitrates provide relief. Treatment focuses on addressing the underlying cardiac ischemia, with vasodilators like nitroglycerin and revascularization procedures as preferred modalities. Cardiac cephalalgia, though rare, necessitates increased clinical awareness to prevent missed diagnoses of acute cardiac ischemia. Its recognition, especially in patients with cardiovascular risk factors presenting with atypical headaches, is vital for timely intervention and improved patient outcomes.