Limb ischemia is most often associated with atherosclerosis and older age. When a younger patient without risk factors for atherosclerosis presents with symptoms of limb ischemia, vascular occlusion may not be suspected initially, thus delaying diagnosis and treatment. Delayed diagnosis can lead to a poor outcome. Here, we describe several uncommon causes of limb ischemia and their initial presentations, workup, and treatment to help guide the practitioner in making a timely diagnosis in this unusual patient population.