Lipomatous hypertrophy of the interatrial septum is a benign condition that must be distinguished from other space-occupying lesions of the atria. Patients with this disorder generally have chronic pulmonary disease and thus are difficult to image with conventional transthoracic two-dimensional echocardiography. Transesophageal echocardiography can provide high quality imaging of intracardiac structures in patients who lack adequate transthoracic echocardiographic windows as a result of pulmonary disease. This case report describes the appearance of lipomatous hypertrophy of the interatrial septum as investigated by transesophageal echocardiography.