Vascular arterial compression syndromes are uncommon disorders due to dynamic anatomic compression of an artery resulting in significant ischemia in the supplied territories with ensuing symptoms. The diagnosis of these disorders requires heightened awareness and a high index of suspicion by the clinician. These diagnoses should be particularly suspected in young patients with typical symptoms but without underlying cardiovascular risk factors. Physical examination may be unremarkable, although the clinical symptoms or signs may be elicited with provocative maneuvers. The diagnostic work-up may include noninvasive tests such as Doppler waveforms, duplex ultrasonography, computed tomographic angiography, and MRI/angiography, but may require conventional angiogram for confirmation or treatment planning. Although provocative maneuvers may indicate arterial compression in symptomatic patients, it is important to note that these maneuvers may also be positive in normal asymptomatic subjects. These disorders are typically treated surgically with procedures leading to decompression, but anticoagulation, thrombolytic therapy, and even surgical bypass operations may be needed for arterial occlusions.