Levoatriocardinal vein (LACV) is characterized by an abnormal connection between pulmonary and systemic venous return. This extremely rare cardiac malformation is usually associated with left-sided obstructive lesions including mitral atresia, hypoplastic left-heart syndrome, and abnormal pulmonary venous connection. Patients may have low systemic cardiac output and pulmonary venous obstruction symptoms. In this manuscript, we report a case with LACV and normal pulmonary venous return with absence of any intracardiac pathology. LACV was demonstrated with echocardiography, angiography, and computed tomography. Surgical correction was made successfully.