We report a case of spontaneous intramural left atrial hematoma with chest pain and pulmonary edema as the primary clinical manifestations. Echocardiographic techniques revealed obstruction of the left atrial cavity by cyst-like masses attached to the posterior and anterior atrial wall. A large intramural hematoma due to extensive atrial wall dissection was observed by magnetic resonance imaging. Surgery confirmed the diagnosis of intramural left atrial hematoma with no complications. We review the clinical and diagnostic profile of the three cases of spontaneous intramural left atrial hematoma reported in the world medical literature.