Abstract Objectives Myxomas are the most common primary tumors of the adult heart and should be considered in the differential diagnosis of intracavitary cardiac masses, along with thrombi and vegetations. Methods We report a case of a 64-year-old obese, hypertensive woman, who presented with dyspnea on moderate exertion as well as fatigue. The patient was admitted to our hospital after the discovery on transthoracic echocardiography of an intracardiac mass, which was highly suggestive for a left atrial myxoma. Results On admission, the patient presents no abnormalities on physical exam, with blood tests showing only slight deviations in the lipid profile. The ECG presents a normal sinus rhythm of 80/min with two premature ventricular contractions and a premature atrial contraction. Transthoracic echocardiography revealed the presence of a well-defined, pediculated, left atrial mass attached to the interatrial septum, measuring 25/17 mm, with no apparent interference in mitral valve motion. We supplemented our investigations with the use of transesophageal echocardiography which further defined the dimensions and location of the tumor, while also showing the presence of an atrial septal aneurysm associated to two small atrial septal defects measuring 6 mm and 7 mm respectively, with a left to right shunt. Cardiac CT examination confirmed the benign aspect of the mass and offered important anatomical elements, useful in preoperative planning. The patient was, therefore, admitted to our Cardiovascular Surgery department, where a successful surgical resection of the tumor was performed, without any local or systemic complications. The differential diagnosis of this mass included intracavitary thrombi, vegetations, as well as other histological subtypes of benign or malignant tumors, while the presence of an atrial septal defect, which represents a possible source of paradoxical emboli, provided an additional challenge. Conclusions This complicated case stands out due to its particular echocardiographic appearance and emphasizes the importance of a comprehensive evaluation in both the differential diagnosis and preoperative assessment.