HMSN is a hereditary symmetric ascending neuropathy of the peripheral nerves. Several cases of HMSN with motility disorders of both eyes have been reported in the literature. We describe a patient with HMSN type 1 and an incomplete peripheral 3rd nerve palsy of the right eye. To the best of our knowledge, this is the first report of developed unilateral oculomotor disorder in a patient with HMSN.