In this case report, we present two novel compound heterozygous ZNF469 mutations causing a mild form of brittle cornea syndrome (BCS) that shows clinical resemblance with the extraskeletal manifestations of osteogenesis imperfecta (OI) type I. The phenotype was not only compatible with features of OI but primarily with EDS. The gene discussed is ZNF469; the disease is Ehlers-Danlos syndrome.