Bertolotti’s Syndrome is defined as chronic back pain caused by transitional lumbosacral vertebra. The transitional vertebra may present with numerous clinical manifestations leading to a myriad of associated pain types. The most common is pain in the sacroiliac joint, groin, and hip region and may or may not be associated with radiculopathy. Diagnosis is made through a combination of clinical presentations and imaging studies and falls into one of four types. The incidence of transitional vertebra has a reported incidence between 4 and 36%; however, Bertolotti’s Syndrome is only diagnosed when the cause of pain is attributed to this transitional anatomy. Therefore, the actual incidence is difficult to determine. Initial management with conservative treatment includes medical management and physical therapy. Injection therapy has been established as an effective second line. Epidural steroid injection at the level of the transitional articulation is effective, with either local anesthetics alone or in combination with steroids. Surgery carries higher risks and is reserved for patients failing previous lines of treatment. Options include surgical removal of the transitional segment, decompression of stenosed foramina, and spinal fusion. Recent evidence suggests that radiofrequency ablation (RFA) around the transitional segment may also provide relief. This manuscript is a comprehensive review of the literature related to Bertolotti’s Syndrome. It describes the background, including epidemiology, pathophysiology, and etiology of the Syndrome, and presents the best evidence available regarding management options. Bertolotti’s Syndrome is considered an uncommon cause of chronic back pain, though the actual incidence is unclear. Most evidence supporting these therapies is of lower-level evidence with small cohorts, and more extensive studies are required to provide strong evidence supporting best practices.