Myxomas are the most common primary benign cardiac tumor. In general, myxoma has a benign growth and chronic entity, so symtoms of myxoma are vague and mild. Therefore the association of an atrial myxoma with acute pulmonary edema and syncope appears to be very rare. Even though very rare, detection of myxoma is important because it is usually fatal in several cases if unrecognized and untreated. We experienced a case of left atrial (LA) myxoma presenting as acute pulmonary edema and syncope of unknown cause in 47 years old female patient. A mass located on left atrium was excised en bloc, and the patient recovered uneventfully. We report the case along with literature review.