Acute painful pupil-involving third nerve palsy requires urgent investigation because it can be due to third nerve compression by a rapidly enlarging aneurysm of the posterior communicating artery. In this chapter, we begin by reviewing the clinical features of third nerve palsy. We next review the common causes of isolated third nerve palsy, which include microvascular ischemia, trauma, compression by neoplasm, and compression by aneurysm. We then discuss the workup of third nerve palsy and review the vascular imaging options to evaluate for aneurysm. Lastly, we discuss the management approach for third nerve palsy due to aneurysmal compression and microvascular ischemia.