Intramural hematoma (IMH) of the aorta is a well-known variant of aortic dissection; however, the optimal initial treatment strategy for type A IMH remains controversial. An English language search of Medline for manuscripts on the treatments and outcomes of IMH with the keywords ‘intramural hematoma’, ‘ascending aorta or type A’, ‘aortic disease’ with cross-references was performed for articles dating from January 1986 to September 2006. Primary outcomes of interest were initial treatment strategies as well as the early and overall mortality rates. Earlier publications studying overlapping patient groups from the same institutions were excluded. Case reports and small series of less than 10 patients were not enrolled. Data from 328 reported cases in 12 studies were extracted. Initial surgery and medical treatment were performed for 168 (51.2%) and 160 (48.8%) patients, respectively. Nine out of 12 studies (75%) came from Asia. The early mortality rate was 10.1% (17/168) and 14.4% (23/160) in patients who received initial surgery and medical treatment, respectively (p = 0.37). The optimal initial treatment strategy for type A IMH may still be individualized. Initial medical treatment and timed surgical therapy seems to be associated with higher early mortality rates in patients with type A IMH, even in a primarily Asian cohort. The impact of either initial treatment strategy on long-term survival must be evaluated in further study.