Intramural hematoma (IMH) is considered a part of acute aortic syndromes (AAS), a group of life-threatening aortic diseases with a similar presentation that appears to have different clinical manifestations and pathological and survival characteristics. AAS comprises three major entities, namely, aortic dissection (AD), IMH, and PAU. IMH-like classic AD is classified using Stanford and DeBakey classification systems to indicate the aortic area involved. Early diagnosis and treatment of AAS are crucial for survival; however, diagnosis of IMH may be delayed and challenging due to atypical presentation, investigation findings, and case progression. In this report, we describe a case of delayed and challenging diagnosis of a Stanford type A IMH that was managed surgically with a good outcome.