Idiopathic carpal tunnel syndrome (CTS) is a common complaint, reflecting entrapment neuropathy of the upper extremity. CTS produces symptoms similar to those of other conditions, such as cervical spondylosis or ischemic or neoplastic intracranial disease. Because of these overlaps, patients with CTS are often referred to a neurosurgeon. Surgical treatment of CTS was started recently in our department. Through this experience, we realized that neurosurgeons should have an increased awareness of this condition so they can knowledgeably assess patients with a differential diagnosis that includes CTS and cervical spinal and cerebral disease. We conducted a literature review to gain the information needed to summarize current knowledge on the clinical, pathogenetic, and therapeutic aspects of CTS. Because the optimal diagnostic criteria for this disease are still undetermined, its diagnosis is based on the patient’s history and physical examination, which should be confirmed by nerve conduction studies and imaging modalities such as magnetic resonance imaging and ultrasonography. Treatment methods include observation, medication, splinting, steroid injections, and surgical intervention. Understanding the clinical features and pathogenesis of CTS, as well as the therapeutic options available to treat it, is important for neurosurgeons if they are to provide the correct management of patients with this disease.