Thoracic outlet syndrome is an important cause of shoulder pain and dysfunction due to compression of neurovascular structures as they traverse the thoracic outlet. Symptoms are most commonly due to compression of the brachial plexus called neurogenic thoracic outlet syndrome (nTOS). Throwing athletes are at increased risk of nTOS because of a variety of biomechanical factors. However, because nTOS symptoms are often nonspecific, delayed diagnosis is common. Neurogenic thoracic outlet largely remains a diagnosis of exclusion with advanced imaging ruling out vascular involvement and diagnostic injections gaining favor in helping localize sites of compression. Although rehabilitation alone may improve symptoms in some athletes, many require surgical treatment for long-term relief. This generally entails decompression of the thoracic outlet by some combination of muscle release, brachial plexus neurolysis, and first rib resection. Outcomes tend to be successful in athletes with most achieving resolution of symptoms and return to athletic activity. NTOS is an important cause of shoulder pain and dysfunction in throwing athletes. The history and physical examination should focus on activities that exacerbate symptoms. Treatment of nTOS generally requires surgical intervention and allows throwing athletes to return to sport.