Purpose To analyze the morphology and density of corneal epithelial cells, keratocytes, and subbasal nerves, in patients with early stage Fuchs' endothelial corneal dystrophy (FECD) by in vivo confocal microscopy (IVCM). Methods IVCM (Confoscan 4, Nidek, Inc.) of the central cornea was performed in 30 corneas of 30 patients with early stage FECD and 13 corneas of 13 normal controls. Images were analyzed for morphology and density of the superficial and basal epithelial cells, keratocyte density, endothelial cell density (ECD), as well as subbasal corneal nerve parameters. Central corneal thickness (CCT) was measured in all patients and normals by ultrasound pachymetry. Results The ECD was significantly lower ( À 45.5%, Po0.001) in FECD patients as compared with controls. Total number of nerves and main nerve trunks were significantly reduced ( À 46.3%, Po0.001; À 39.7%, Po0.001) in patients with FECD. Posterior keratocyte density was significantly higher in FECD patients (Po0.001). Significant inverse correlations were found between CCT and total number of nerves (r ¼ À 0.69, Po0.001), CCT and main nerve trunks ( À 0.47, P ¼ 0.016), as well as CCT and total nerve length (r ¼ À 0.62, P ¼ 0.006). Significant correlation was found between ECD and total number of nerves (r ¼ 0.44, P ¼ 0.012) as well as between ECD and main nerve trunks (r ¼ 0.65, Po0.001). Conclusions IVCM demonstrates alterations in corneal innervation in patients with early stage FECD, suggesting a potential role of corneal nerves in the pathogenesis of FECD. Additional studies are required to investigate whether subbasal nerve alterations are caused by nonspecific corneal edema, from FECDinduced decrease in ECD, or potentially leading to loss of endothelial cells.