Primary congenital ectropion uveae is an extremely rare ocular malformation frequently associated with unilateral glaucoma. We report on a 15-year-old boy with unilateral congenital ectropion uveae, glaucoma and transvitreal strands in an optically empty vitreous. Dark adaptation was normal, but scotopic ERG showed subnormal b-wave amplitudes in the affected eye, which is a typical finding in hereditary vitreoretinal degenerations. The coincidence of primary congenital ectropion uveae and unilateral vitreoretinal degeneration without a family history seems to be sporadic and very extraordinary but could be due to a common defect of maturation.