Objective To observe the retinal manifestations and classification of mitochondrial encephalomyopathy，and explore the relationship between retinopathy and systemic manifestations.Method The clinical data of 88 inpatients with mitochondrial encephalomyopathy were retrospectively analyzed，in whom 12 patients（24 eyes）with retinal manifestations who diagnosed by ophthalmology consultation and complete medical records were collected. There were nine males and three females aged from 14 to 33 years with the mean age of（20.1±7.0）years. The disease duration ranged from 2.5 to 20 years，with the mean of（9.5±6.8）years. All the patients had the eye symptoms of the different degree，such as limbs weakness，hearing decline and central nervous system symptoms. Ophthalmologic examination including best corrected visual acuity，slit lampa microscope，indirect ophthalmoscopy，noncontact Tonometer，ptosis，ocular movement，pupillary reflex and color fundus photography. Among the patients，three，one，two and five patients had undergone fundus fluorescein angiography（FFA），optical coherence tomography（OCT），lectroretinogram（ERG）and visual field examination respectively. Diabetic retinopathy were divided into “salt and pepper”， retinitis pigmentosa（RP），retinal pigment epithelium（RPE），choroidal capillary atrophy and simplex optic atrophy according to the inspection results.Results All the patients′ both eyes were involved，the disease degree of bilateral eyes was accordant. The ptosis and（or）eye movement limitation were found in nine patients（75.0%），and decreased visual acuity was in six patients（50.0%）.“Salt and pepper” was found in six patients（12 eyes），presenting retinal granular pigmentation and depigmentation；the visual acuity was 0.4-1.2；no central nervous system symptoms were found in patients，such as hearing decline，twitch，ataxia and hypophrenia. RP was found in one patient（two eyes），presenting retinal cells sample pigmentation，retinal vessel shrink，optic atrophy；the vision were light perception in both eyes；hypophrenia，hearing decline，bilateral lower limbs pain and onset twitch were also found in them. RPE and choroidal capillary atrophy were found in three patients（six eyes），the choroidal great vessels and flake pigment accumulation surrounding the retina were observed；the visual acuity was hand movement0.7；limbs weakness was found in two patients；hearing decline was found in three patients；barylalia and hypophrenia were found in two patients；somnolence was found in one patient. Simplex optic atrophy was found in two patients（four eyes）；the vision was 0.1-0.7；central nervous system symptoms were found in patients，such as limbs weakness，twitch，hypophrenia and headache.Conclusion Retinopathy types is concerned with visual prognosis and central nervous system symptoms.