Objective To observe the optical coherence tomography (OCT) features of Best vitelliform macular dystrophy (BVMD) at different stages.Methods Twenty-eight BVMD patients (56 eyes) were enrolled in this study. All the patients were examined for visual acuity, slit-lamp microscopy, direct ophthalmoscope, fundus photography, electrooculogram, fundus fluorescein angiography (FFA) and OCT. Fifty-six eyes were classified into stage 0 (eight eyes)、Ⅰ (two eyes)、Ⅱ(10 eyes)、Ⅱa （12 eyes）、Ⅲ (six eyes) 、Ⅳa (six eyes)、Ⅳb (five eyes) and Ⅳc (seven eyes) accordingly. The OCT features of BVMD at different stages were observed.Results The OCT results showed that the macular area was normal in eyes of stage 0; disturbance of retinal pigment epithelium (RPE) and subretinal hyporeflective area were found in eyes of stage I; the location of the yellowish material between RPE and the inner segment and outer segment (IS/OS) with normal appearance in RPE and IS/OS interface were found in eyes of stage Ⅱ. In all the other progressing stages from Ⅱa、Ⅲ and Ⅳ, the vitelliform material appeared as a thicker highly reflective lesion located between the outer nuclear layer and RPE layer, usually accompanied by optical hyporeflective lesion. Images of stage Ⅳc were in similar appearance besides edema of retina. OCT images of Ⅳb stage were demonstrated atrophy of retinal layer and IS/OS loss with fibrosis. Conclusions OCT demonstrated the location of the yellowish material between RPE and IS/OS. Optical hyporeflective lesion between the outer nuclear layer and RPE layer accompanied thicker highly reflected lesion might be the characteristic image in stages II a to IV of BVMD.