The celiac axis (CA) and its branches are critically important arteries that supply blood to the vital solid and hollow abdominal viscera of the foregut. There are many potential anatomic configurations, with up to half the population having a variation from the classic pattern of the CA bifurcating into the hepatosplenic trunk and left gastric artery. These configurations result from permutations in the fusion of the paired dorsal aortas during the first trimester. Despite the short length of the CA, it is affected by a wide range of pathologic conditions, including mesenteric ischemia due to intrinsic occlusion (secondary to causes such as atherosclerosis or thromboembolic events) and extrinsic compression from masses or the median arcuate ligament. Symptoms of mesenteric ischemia are nonspecific and include postprandial abdominal pain and weight loss; thus, the underlying pathologic condition may be found only when being sought specifically. More unusual pathologic conditions include dissection, aneurysms, and vascular malformations. Awareness of the pathologic conditions that affect the CA is important for both diagnostic and interventional radiologists. Early recognition and treatment of CA disease may prevent catastrophic hemorrhage and bowel infarction. Both endovascular and surgical approaches to treatment are greatly enhanced by correct identification of arterial anatomic variants; catheter angiography, computed tomographic angiography, and magnetic resonance angiography can facilitate detection of these variants. Knowledge of the different anatomic permutations is essential to guide endovascular procedures, such as hemorrhage control, transarterial interventional oncologic therapy, and treatment of visceral artery aneurysms. Online supplemental material is available for this article.