Imaging diagnosis of nonatheromatous carotid artery disease is challenging due to its low prevalence in contrast to that of atheromatous disease. Congenital anomalies are frequently discovered incidentally, as the chronicity of these conditions allows for compensatory flow development. The inflammatory conditions typically present with nonspecific courses, and a high clinical suspicion along with timely imaging evaluation can guide the diagnosis. Carotid dissection is the result of a partial disruption of the arterial wall and can be seen in previously healthy patients, in patients with underlying noninflammatory arteriopathies or trauma. Traumatic injuries to the carotid artery may occur under many different conditions and mechanisms and timely recognition of high-risk patients improves patient outcomes. Although free-floating thrombi (FFT) formation is typically seen with atherosclerotic plaque rupture, different conditions may also predispose to FFT. In this review article, we study the different imaging features of nonatheromatous carotid artery disease using ultrasonography, computed tomography angiography, magnetic resonance angiography, and digital subtraction angiogram.