BACKGROUND Aneurysm compression, diabetes, and traumatic brain injury are well-known causative factors of oculomotor nerve palsy (ONP), while cases of ONP induced by neurovascular conflicts have rarely been reported in the medical community. Here, we report a typical case of ONP caused by right posterior cerebral artery (PCA) compression to increase neurosurgeons' awareness of the disease and reduce misdiagnosis and recurrence. CASE SUMMARY A 54-year-old man without a known medical history presented with right ONP for the past 5 years. The patient presented to the hospital with right ptosis, diplopia, anisocoria (rt 5 mm, lt 2.5 mm), loss of duction in all directions, abduction, and light impaired pupillary reflexes. Magnetic resonance angiography and computed tomography venography examinations showed no phlebangioma, aneurysm, or intracranial lesion. After conducting oral glucose tolerance and prostigmin tests, diabetes and myasthenia gravis were excluded. Cranial nerve magnetic resonance imaging showed that the right PCA loop was in direct contact with the cisternal segment of the right oculomotor nerve (ON). Microvascular decompression (MVD) of the culprit vessel from the ON through a right subtemporal craniotomy was carried out, and the ONP symptoms were significantly relieved after 3 mo. CONCLUSION Vascular compression of the ON is a rare pathogeny of ONP that may be refractory to drug therapy and ophthalmic strabismus surgery. MVD is an effective treatment for ONP induced by neurovascular compression.