Compression of the neurovascular structures at the level of the scalene triangle and pectoralis minor space is rare, but increasing awareness and understanding is allowing for the treatment of more individuals than in the past. We outlined the recognition, preoperative evaluation, and treatment of patients with neurogenic thoracic outlet syndrome. Recent work has illustrated the role of imaging and centrality of the physical examination on the diagnosis. However, a fuller understanding of the spatial biomechanics of the shoulder, scalene triangle, and pectoralis minor musculotendinous complex has shown that, although physical therapy is a mainstay of treatment, a poor response to physical therapy with a sound diagnosis should not preclude decompression. Modes of failure of surgical decompression stress the importance of full resection of the anterior scalene muscle and all posterior rib impinging elements to minimize the risk of recurrence of symptoms. Neurogenic thoracic outlet syndrome is a rare but critical cause of disability of the upper extremity. Modern understanding of the pathophysiology and evaluation have led to a sounder diagnosis. Although physical therapy is a mainstay, surgical decompression remains the gold standard to preserve and recover function of the upper extremity. Understanding these principles will be central to further developments in the treatment of this patient population.