Since the processing of full-length Gli3, occurs in primary cilia, syndromes known as ciliopathies, in which cilia function/structure is compromised, include polydactyly as part of their spectrum of defects—examples being, Bardet-Biedl syndrome (BBS–OMIM 209900, Forsythe and Beales, 2013) and Meckel-Gruber syndrome (OMIM 249000, Shaheen et al., 2013). Here, GLI3 is linked to Bardet-Biedl syndrome.