OBJECTIVE The tick-borne spirochete Borrelia burgdorferi sensu lato can cause several neural manifestations from the peripheral and central neural system. There are several case reports in the literature of optic nerve involvement in association with Lyme neuroborreliosis, but clinical guidelines as to when Lyme disease should be considered in optic neuropathy is lacking. RESULTS Papilledema caused by raised intracranial pressure in Lyme meningitis seems mainly to affect children, although some adult cases have been reported. Very few cases of retrobulbar optic neuritis, papillitis, neuroretinitis and ischemic optic neuropathy have shown evidence of a strong association with Lyme neuroborreliosis. CONCLUSIONS Optic neuropathy in Lyme neuroborreliosis is rare. The cases reported in the literature are not sufficient for making a list of clinical 'red flags'. However, in adult cases, special attention seems reasonable in patients with painless visual loss, bilateral optic nerve head swelling with or without an elevated cerebrospinal fluid opening pressure. In endemic areas, any optic neuropathy may still be considered for a Lyme neuroborreliosis work-up. The use of accepted criteria for establishing the diagnosis of Lyme neuroborreliosis is emphasised.