Hand surgeons routinely treat carpal and cubital tunnel syndromes, which are the most common upper extremity nerve compression syndromes. However, more infrequent nerve compression syndromes of the upper extremity may be encountered. Because they are unusual, the diagnosis of these nerve compression syndromes is often missed or delayed. This article reviews the causes, proposed treatments, and surgical outcomes for syndromes involving compression of the posterior interosseous nerve, the superficial branch of the radial nerve, the ulnar nerve at the wrist, and the median nerve proximal to the wrist.