Although worldwide, autosomal recessive OI is rare, the frequency of a form of OI III is relatively high in the indigenous Black African population of South Africa.6–8 When molecular investigatios have been undertaken, mutations in the FKBP10 gene that encodes the collagen chaperone-like protein FKBP65 were identified.9FKBP10 is one of the newer members of an expanding list of AR OI genes and was first documented in OI III by Alanay et al.10 The gene discussed is FKBP10; the disease is osteogenesis imperfecta.