Disorders of the third, fourth, and sixth cranial nerves are common in neurology. Isolated palsies of each of these nerves have signs that can assist in localizing damage to the nucleus, fascicle, or the nerve in its subarachnoid or intracavernous portion, and each varies in the frequency of the different underlying pathologies. The distribution of causes of pediatric ocular motor palsies also differs from that in adults and includes some characteristic developmental syndromes, such as Duane retraction syndrome. Diffuse involvement of the ocular motor nerves is seen in Miller-Fisher syndrome, and multiple palsies indicate a cavernous sinus syndrome, which has several causes. Internuclear ophthalmoplegia is a distinct sign of damage to the medial longitudinal fasciculus, most commonly due to ischemia or demyelination.