We report on a consanguineous black South African family with type I MCD, which is caused by a novel E71Q mutation in CHST6. It is the first report of MCD in a Sub-Saharan African family, although it is not the first report of MCD in an individual with African ancestry as Patel et al. previously identified a novel mutation in CHST6, which is associated with MCD type II in an African American [40]. The gene discussed is CHST6; the disease is macular corneal dystrophy.