Here, we demonstrated an unusual R124C mutation in TGFBI associated with the RBCD, which was a mutation known to be responsible for corneal lattice dystrophy type I. Therefore, along with G623D and R124L, the R124C mutation in TGFBI is also found to be responsible for RBCD. The gene discussed is TGFBI; the disease is lattice corneal dystrophy type I.