Acute aortic syndrome includes classic aortic dissection, aortic intramural hematoma, and penetrating atherosclerotic ulcer – a group of conditions that are defined by their dynamic evolution and similar clinical manifestation. Accurate diagnosis and prompt treatment are essential as all the aforementioned conditions are a significant threat to a patient’s life. However, acute aortic syndrome and especially aortic intramural hematoma may be challenging diagnostic problems. Intravascular ultrasound imaging is a diagnostic method that can be useful for more thorough evaluation of the aortic lesion and can particularly aid in discerning the different forms of acute aortic syndrome. We present a case of a patient with aortic intramural hematoma that was missed by conventional imaging studies but was successfully visualized with intravascular ultrasound imaging.