As our understanding of the anatomy of the ulnar tunnel has increased, so too has our ability to clinically predict the specific sites of compression in ulnar tunnel syndrome. Anatomic studies have described in detail the course of the ulnar nerve as it passes through the ulnar tunnel and have helped correlate symptoms with anatomic location. Although the most common cause of compression is from a ganglion, other space-occupying lesions, such as tumors, anomalous muscles, or a thrombosed ulnar artery, are important to consider in the initial evaluation of the patient. While conservative management can sometimes be successful, the mainstay of treatment of this condition remains meticulous surgical exploration and decompression.