Total situs inversus associated with the oculoauriculo‐vertebral spectrum

A patient is reported with the association of the oculo-auriculo-vertebral spectrum and situs inversus. Conditions with overlapping features and previously reported conditions associated with the oculo-auriculo-vertebral spectrum are reviewed. These conditions, as well as the reported association of the oculo-auriculo-vertebral spectrum and situs inversus can probably be seen as a part of the mesodermal malformation spedtrum, due to an early abnormality in the development of the primitive streak or embryonic mesoderm.