This paper describes a case of a child with Poland-Möbius syndrome in the context of a maternally inherited PLXND1 gene mutation, a gene that is considered a candidate gene for Möbius syndrome but has hitherto not been described in the context of either Poland syndrome or Poland-Möbius syndrome, despite multiple case reports suggesting a nosologic link between the two syndromes. This evidence concerns the gene PLXND1 and Mobius syndrome.