Background: Information regarding the treatment of high-grade spondylolisthesis (HGS) in adults has been previously described; however, previous descriptions of the evaluation and surgical management of HGS do not represent more recent and now established approaches. The purpose of the current review is to discuss current concepts in the evaluation and management of patients with HGS. Methods: Literature review. Results: HGS is diagnosed in up to 11.3% of adults with spondylolisthesis and typically presents as nonspecific lower back pain. Regarding evaluation, a thorough history and physical examination should be performed, which may help predict the presence of HGS. Diagnostic imaging, and specifically the use of spino-pelvic parameters, are now commonly implicated in guiding treatment course and prognosis. When surgical intervention is indicated, surgical approaches include in situ fusion variations, reduction and partial reduction with fusion, and vertebrectomy. Although the majority of studies suggest improvements with these approaches, the literature is limited by a low level of evidence with regards to the superiority of one technique when compared with others. Conclusions: HGS is a unique cause of low back pain in adults that carries considerable morbidity, but rarely presents with neurologic symptoms. Although the definitions, classifications, and methods of diagnosis of this spinal deformity have been established and accepted, the ideal surgical management of this deformity remains highly debated. Fusion in situ techniques are often technically easier to perform and provide lower risk of neurologic complications, whereas reduction and fusion techniques offer greater restoration of global spino-pelvic balance. Preoperative spinopelvic parameters may have utility in assisting in procedural selection; however, future, higher-quality and longer-term studies are warranted to determine the optimal surgical intervention among the widely available techniques currently used, and to better define the indications for these interventions.