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Abstract Atrial intramural hematoma is a rare entity. It was reported to occur spontaneously especially in patients with recent history of cardiac surgery or cardiac trauma. In this report, a case of spontaneous intramural hematoma associated with large bloody stained pericardial effusion presented with progressive shortness of breath as the primary clinical manifestation is described. The diagnosis was tentatively achieved by transesophageal echocardiography and cardiac computed tomography scan and was later confirmed during surgery. The patient recovered uneventfully following surgery and follow-up echocardiography demonstrated complete resolution of pre-existing hematoma. < Learning objective: We present a case of spontaneous intramural atrial hematoma and cardiac tamponade in a 61-year-old patient with a history of progressive shortness of breath. The proper diagnosis and surgical intervention are described. This case description will help increase the awareness regarding the diagnosis of this rare cardiac condition.>