To analyze the clinical characteristics of cardiac cephalalgia (CC) and improve the understanding and diagnosis accuracy of this disease. Methods: A case of CC with autonomic symptoms from our hospital was reported. Medical literature from January 1997 to October 2021 was searched for patients diagnosed with CC, and the findings were summarized. Results: The patient in this case report presented bilateral temporoparietal pulsating headaches and a visual analog scale (VAS) score of 7～8 accompanied by nausea, photophobia, phonophobia, chest tightness, dyspnea, and autonomic symptoms such as salivation, tearing, and runny nose. Homomorphic ECG showed rapid rhythm-dependent extensive myocardial ischemia during headache attacks. Coronary artery CTA suggested three-vessel disease. Anti-angina pectoris treatment was effective. Forty-five cases of CC were reviewed. The clinical manifestations were mainly activity-induced, moderate to severe headaches in various locations, mostly pulsating and compressive, and accompanied by chest discomfort, nausea, palpitations, and sweating. The duration was mostly under 1 hour, and symptoms were relieved by rest and nitroglycerin. Conclusion: CC is a rare disease. Patients over 50 years old with activity-induced, moderate to severe headache combined with typical cardiovascular disease risk factors should be highly suspected of having CC. Early diagnosis and anti-angina therapies could contribute to good prognosis.