Fuchs endothelial corneal dystrophy (FECD) is the most common primary corneal endothelial dystrophy and the leading indication for corneal transplantation worldwide. FECD is characterized by a progressive decline in the number and function of corneal endothelial cells (CECs) and the formation of guttae. New technologies enable more accurate classification and staging of the disease to tailor treatment. The selective replacement of the endothelium and Descemet membrane through the development of advanced surgical techniques including Descemet membrane endothelial keratoplasty (DMEK) has improved postoperative outcomes. While DMEK remains a standard therapeutic option for patients with FECD, newer modalities such as Descemet’s stripping only with or without Rho-associated protein kinase Inhibitors and cell-based therapies are emerging. Since FECD patients often have cataracts, it is important to identify the predominant cause of visual loss and determine the timing and sequence of cataract surgery and keratoplasty.