BACKGROUND AND AIMS: Extraintestinal manifestations of inflammatory bowel disease (IBD) can involve the orbit and the optic nerve. Although these manifestations are rare, they can be particularly serious as they can lead to permanent loss of vision. The aim of the review is to present the existing literature on IBD-related optic nerve and orbital complications. METHODS: A literature search identified the publications reporting on incidence, clinical features and management of IBD patients with optic nerve and orbital manifestations. RESULTS: Posterior scleritis and orbital inflammatory disease (orbital pseudotumor) are the most commonly encountered entities affecting the structures of the orbit. On the other hand, the optic nerve of IBD patients can be affected by conditions such as optic (demyelinating) neuritis ("retrobulbar" neuritis), or ischaemic optic neuropathy. Other neuro-ophthalmic manifestations that can be encountered in patients with IBD are related to increased intracranial pressure or toxicity secondary to anti tumour necrosis factor (anti-TNF) agents. CONCLUSIONS: IBD-related optic nerve and orbital complications are rare but potentially vision-threatening. Heightened awareness and close cooperation between gastroenterologists and ophthalmologists are warranted.