Multidisciplinary approach of assessing malformed fetuses exemplified in a rare case of pentalogy of Cantrell associated with craniorachischisis, pulmonary extrophy and right-sided aortic arch with aberrant brachiocephalic artery.

We present the imagistic and pathological assessment of a unique case of complete pentalogy of Cantrell associated with craniorachischisis, pulmonary extrophy and right-sided aortic arch with aberrant brachiocephalic artery. For this particular case, the complete and correct diagnosis required a post-mortem imagistic high-resolution magnetic resonance imaging (MRI) at 7 T and detailed stereomicroscopic autopsy. Also, we discussed the pathogenesis and possible etiology of pentalogy of Cantrell and the associated malformations of the case presented.