Cardiac myxoma is the most common primary benign cardiac tumor (up to 50% of all primary cardiac neoplasms). The implementation of the modern imaging techniques into the clinical practice, particularly, 2D echocardiography, computed tomography and magnetic resonance tomography (MRI) results to the prompt diagnosis of the myxoma. However, the absence of specific clinical features, insufficient awareness of this condition among the physicians along with a rare prevalence, may lead to a misdiagnosis. This case report is notable for the relatively late diagnosis of a giant left atrial myxoma due to a number of circumstances but with successful surgical treatment.