Oculomotor nerve plays an important role in regulating the function of eyeball by controlling levator palpebrae superioris muscle, superior rectus muscle, inferior rectus muscle, medial rectus muscle, inferior oblique muscle, sphincter pupillae muscle and ciliary muscle. Oculomotor nerve palsy is a common clinical symptom characterized by diplopia, ptosis, ophthalmoplegia or pupillary dysfunction. The etiologies of oculomotor nerve palsy are complicated, including intracranial aneurysm, cerebral infarction and hemorrhage, diabetes, intracranial inflammation, craniocerebral trauma, congenital disease and so on. Palsy caused by different etiologies has corresponding clinical features. In this review, we summarized the common causes of oculomotor nerve palsy, and their corresponding pathogenesis, clinical manifestations, signs, treatments and prognosis, hoping to provide help for rapid identification of etiology and effective treatment of the oculomotor nerve palsy.