The aim of this study is to present rare retinal disease of unknown origin.27-years-old man was diagnosed because of poor vision in the left eye, which lasted 4-5 weeks. Following examinations were performed: Visual acuity, contrast sensitivity, color perception, visual field, fluorescein angiography (AF), visual evoked potentials (VEP), full-field flash electroretinography (flash ERG), focal-foveal electroretinography (focal ERG), multifocal electroretinography (multifocal ERG). Follow-up 1.5 year.Visual acuity of right eye was 1.25 (-0.1 log MAR) and left eye 1.0 (0.0 log MAR). In left eye between optic disc and macula irregular lesion with dots of retinal pigment epithelium atrophy and little edema was seen. AF revealed small "window" defects. In visual field of the left eye paracentral relative scotoma occurred and in stereokampimetry central relative scotoma was found about 5 degrees from the fixation point. In VEP latency of P100 was delayed and amplitude was reduced in both eyes. In flash as well as focal ERG small reduction of cone function and delayed implicit time of b-wave were found in left eye. In general examination no focal inflammation and no abnormalities in laboratory tests were found. The patient was treated with steroids for 3 weeks. After ten days of general steroid treatment visual acuity improved to 1.25 and subjective improvement of vision occurred. Control examination after 1.5 year revealed no patient's complains, visual acuity 1.25, no change in visual field, VEP improvement. In left eye flat irregular area with pigment epithelium atrophy was seen.Acute macular neuroretinopathy may be diagnosed after detail examination. Prognosis is generally good, recovery is slow, but despite of local retinal atrophy subjective complains disappear completely.