Lyme disease is an emerging new spirochaetal disease in which ocular complications may arise. We have seen a 45-year-old woman who developed unilateral endophthalmitis leading to blindness during the course of this disease. Ocular tissue showed the characteristic spirochete. A literature review shows that the commonest ocular manifestation of Lyme disease is a mild conjunctivitis, but other symptoms may include periorbital oedema, oculomotor palsies, uveitis, papilloedema, papillitis, interstitial keratitis, and others. Ophthalmologists treating patients from Lyme disease endemic areas need to be aware of the protean clinical manifestation of this disease.