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Neurogenic thoracic outlet syndrome is a complex condition and is commonly misunderstood. Historically, much of this confusion has been because of its grouping with other diagnoses that have little in common other than anatomic location. Modern understanding emphasizes the role of small unmyelinated C type pain and sympathetic fibers. Diagnosis is primarily clinical, after ruling out other common conditions. Hand therapy is usually the first-line treatment with variable success. Local anesthetic, botulinum toxins, or steroid injections can aid in diagnosis and offer short-term relief. Although surgery can yield reliable results, it is technically challenging, and the preferred surgical approach is a matter of debate. Despite limitations in diagnosis and treatment, recognition and successful treatment of this condition can be highly impactful for the patient., (Copyright © 2024 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)