Corneal endothelial cell dysfunction is a major cause of corneal edema and vision loss. Its limited regenerative capacity poses significant challenges to clinical treatment. In recent years, transition zone (TZ) of corneal margin has been recognized as a potential source of corneal endothelial stem or progenitor cells. This has opened up a new research direction for cell therapy of corneal endothelial diseases. This article systematically reviews the anatomical localization and ultrastructural characteristics of corneal TZ cells. Their isolation, culture, and in vitro expansion strategies are summarized. Emphasis was placed on summarizing its biological biomarker lineage and its intrinsic association with neural crest / periocular mesenchymal development. Meanwhile, the current key technological challenges and future development directions are discussed around its potential application as seed cells for corneal endothelial regeneration and endogenous therapeutic targets. This article aims to provide theoretical basis for the development of efficient and safe corneal endothelial regeneration treatment strategies. It also provides new research ideas for promoting their clinical translation.