Heterotaxy syndrome is a term used to describe disorders of lateralization of thoracic and abdominal organs as well as atria. It is commonly associated with complex congenital heart disease as well as abnormalities of the spleen, intestinal malrotation, and abnormalities of liver and stomach position. Most reported cases of heterotaxy syndrome are sporadic, though familial occurrences have also been reported. We report a case of monozygotic twins, both with heterotaxy syndrome, complex congenital heart disease, and anatomical features in both twins that are almost mirror images to each other.