We present the case of a patient (a 21-year-old woman) with acute and complete loss vision of the left eye, and severe periodic left ocular and left orbital pain. Visual, acuity right eye = 20/20. Under steroid and nonsteroid general and local treatment, the visual acuity was improved (VA left eye = 0, 1,n.c.) and a central scotoma was developed. Ophthalmoscopic analysis of the left eye showed elevated and blurred optic disk margins, retinal hemorrhages, venous congestion. The diagnosis of borreliosis was based on clinical and ocular findings and determinations of antibodies to Borrelia burgdorferi by enzyme-linked immunosorbent assay and immunoblot analysis, the detection of DNA of B.burgdorferi by polymerase chain reaction and exclusion of other infectious and inflammatory causes (tuberculosis, toxoplasmosis, syphilis and sarcoidosis were excluded). The commonly used blood, urine and spinal fluid tests, cerebral MRI and angiofluorography were also done. The patient received oral ceftriaxone 2gr/daily for 4 weeks, 2 cures and Doxycycline 2 gr/daily, 21 days. Under the treatment, the clinical signs had significantly improved (VA left eye = 20/20), but the central scotoma remained. The case is unusual because only one eye was affected (typically decreased visual acuity occurs on both eyes), other signs of the disease were absent, and the recovery under the antibiotic treatment was excellent.