Isolated renal artery dissection (IRAD) is a rare and often unrecognized clinical entity, with a paucity of data on its epidemiology and management. We extracted 129 cases of IRAD from the medical literature between 1972 and 2016. IRAD as a result of an extended dissection from the aorta and splanchnic or mesenteric arteries was excluded. The mean age of presentation was 42.7±12.9 years, with a male predominance (79%). Abdominal pain (75.9%) was the most common presenting symptom. Etiology was more likely to be spontaneous (76%) than traumatic (12%), iatrogenic (9%), or drug-induced (1.5%). The most common risk factors were hypertension (28.7%), fibromuscular dysplasia (8.5%), and Ehlers-Danlos syndrome (5.4%). Unilateral renal artery dissection (right 45.5%, left 40.5%) was more frequent than bilateral (14%). More than half (56.6%) of the cohort were managed medically (blood pressure control and /or anticoagulation). Of those who underwent intervention, endovascular stenting or embolization (35%) was utilized more frequently than nephrectomy or bypass (21%). Computed tomography (CT) and magnetic resonance angiography (MRA) have the highest diagnostic sensitivity (91% and 93%, respectively) as compared to ultrasonography (27%). A high degree of clinical suspicion is required to diagnose IRAD. CT and MRI have a higher diagnostic sensitivity. As compared to invasive management, conservative management has comparable outcomes.