Objective: To investigate the imaging features of Fuchs endothelial corneal dystrophy (FECD) and to provide imaging evidence for the diagnosis of this disease. Methods: Retrospective case series study. A total of 128 eyes (64 patients, including 19 males and 45 females) diagnosed with FECD at the Beijing Tongren Eye Center of Capital Medical University from January 2014 to December 2016 were enrolled. The average age was 57.8±12.9 years. There were 25 eyes of stage Ⅰ (19.5%), 81 eyes of stage Ⅱ (63.3%), 16 eyes of stage Ⅲ (12.5%) and 6 eyes of stage Ⅳ (4.7%).All patients underwent specular microscopy, and 41 patients (82 eyes) had in vivo confocal microscopy (IVCM). The patients' general data, clinical stage, and image features of specular microscopy and IVCM were analyzed. The enumeration data was compared by chi-square test. Differences of measurement data were compared by ANOVA. Data which cannot be accurately measured was compared by rank sum test. Results: As the disease progressed, the number, incidence rate, and fusing rate of dark"holes"on specular microscopy increased. The number of guttata on IVCM increased, and the fusing pattern of guttata developed from pair-like, chain-like to group-like. On specular microscopy, the mean rank of stage Ⅰ (78.2), stage Ⅱ (228.4), stage Ⅲ (284.5) and stage Ⅳ (288.5) was statistically different (χ²=84.183, P=0.000). All positions of all eyes of stage I had no fusion of the dark "holes". The incidence of fusion on the peripheral cornea gradually increased significantly (χ²=27.167, P=0.000) from stage Ⅱ (45.1%, 146/324), stage Ⅲ (76.3%, 45/59) to stage Ⅳ (83.3%, 15/18). Conclusions: The imaging features of specular microscopy and IVCM can be applied as an important basis for early diagnosis of FECD. Specular microscopy is a practical method for rapid screening of FECD. IVCM is an important imaging basis for clarifying the appearance of guttata and analyzing fusion features, so as to guide the differentiation of stages. (Chin J Ophthalmol, 2020, 56:938-943).