We report 3 members of an extended family who presented with bilateral peripheral corneal edema consistent with Brown-McLean syndrome. On clinical examination, all eyes demonstrated normal central corneas and marked peripheral edema. In vivo confocal microscopy of the peripheral cornea highlighted similar observations in the 6 eyes including endothelial pigmentation, masked stromal structure due to edema, prominent nerves, and localized basal epithelial edema. In the central cornea, in vivo confocal microscopic observations highlighted large cellular structures with prominent nuclei in groups consisting of several cells of similar appearance. In vivo confocal microscopy may enhance the diagnosis of Brown-McLean syndrome and may be used for dynamic evaluation and postoperative follow-up of the structural corneal changes.