Aortic valve myxoma is a rare benign cardiac neoplasm. The association of aortic valve myxoma with cardiogenic shock and acute myocardial infarction has been reported in few observations. We report the case of a 19-year-old male patient, who underwent chest pain for two weeks, then further examinations indicated a soft spherical mass on the left coronary cusp. The patient had sporadic cardiogenic shock and acute myocardial infarction during the preoperative preparation, and we carried out emergency effective cardiopulmonary resuscitation (CPR), followed by emergency surgical operation for aortic valve tumor. Postoperative pathology showed it was a myxoma. The patient recovered smoothly and was discharged on postoperative day 7. Cardiogenic shock and acute myocardial infarction are very nonspecific, and we should be aware that patients with cardiogenic shock and acute myocardial infarction possibly suffer from aortic valve myxoma.