Dissociated vertical deviation (DVD) is an intermittent anomaly of the non-fixing eye. Although association of DVD with sensory visual deprivation owing to congenital or acquired opacities of the ocular media has been reported, its association with congenital hereditary endothelial dystrophy (CHED) has not been reported hitherto. We report a case having a bilateral asymmetric DVD, in a know case of bilateral CHED.