Background: Injuries to the acromioclavicular (AC) joint are common and should be suspected in patients who have shoulder pain in the region of the acromion and clavicle. Injuries to the AC ligament can cause horizontal instability and are often neglected or underdiagnosed, which can lead to poor patient outcomes. Purpose: To perform a systematic review of the literature on the diagnosis and treatment of horizontal instability of the AC joint. Study Design: Systematic review. Methods: The authors performed a systematic review using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. PubMed and EMBASE were searched for studies that investigated diagnosis, treatment, and failure of operative management of acute and chronic AC separations. Studies that did not specifically evaluate AC joint injuries, were not written in English, or were specific only to vertical instability of the AC joint were excluded. Results: Overall, 23 articles met the inclusion criteria and were therefore included in this systematic review. Diagnosing horizontal AC instability is difficult using plain radiographs; dynamic views were shown in some cases to better detect horizontal instability than with static views. More than 60 procedures for treating AC joint injuries have been published, but many focus on vertical rather than horizontal instability. Modifications to current surgical procedures to incorporate reconstruction of the horizontal component showed improved patient outcomes. Such modifications included additional AC joint suture cord cerclage, combined AC and coracoclavicular ligament reconstruction, and the Twin Tail TightRope triple button technique. Failure after surgical stabilization of AC joint separation has been reported to occur in 15% to 80% of cases. Conclusion: No consensus is available regarding the best practices for diagnosis, evaluation, and treatment of acute or chronic horizontal instability of the AC joint. Moreover, horizontal instability injuries are often neglected or poorly understood, making diagnosis difficult, which may lead to high complication rates and failure after surgical stabilization.