A tonic pupil may occur in isolation or as part of a systemic disorder. We report a patient who developed tonic pupils and an abduction deficit in the setting of polyarteritis nodosa. The combination of a tonic pupil and an abduction deficit should suggest the possibility of a vasculopathic disorder, because the ciliary ganglion and lateral rectus muscle are both supplied by the lateral muscular artery. Widespread small artery and arteriolar narrowing and occlusion are the hallmarks of polyarteritis nodosa. Unusual ischemic syndromes may occur, such as this rare combination of neuro-ophthalmic signs, by involvement of both the nutrient artery and its collaterals. We are unaware of other reports of neuropathic tonic pupils in association with polyarteritis nodosa.