Aortic dissection is rare in the pediatric and young adult population. We hereby present a case of a 17-year- old male patient, without any predisposing factors, who developed an aortic dissection. The initial presentation was acute abdominal pain with massive retroperitoneal hematoma. His clinical condition deteriorated rapidly, did not respond to surgical hemostasis, and died within 36 hours of admission. The major autopsy finding was dissection of the descending aorta, extending from 2 cm distal from the origin of the left Subclavian artery. Microscopically, the aortic sections showed intimal thickening and tearing, medial smooth muscle loss, which was replaced by fibrous tissue, fragmentation of elastic lamellae with widening of interlamellar spaces, and cystic medial degeneration. The morphological features represented degenerative changes of the aorta, which were unusual in such a young patient. The pathophysiology, predisposing factors and relevant reports in the literature of aortic dissection in young patients are reviewed.