Mutations in the gene encoding CC2D1A cause a rare form of ID and ASD in humans, and this protein is emerging as a critical regulator of intracellular signaling with roles in cognitive function (Basel-Vanagaite et al., 2006; Manzini et al., 2014), immunity (Zhao et al., 2010; Chang et al., 2011) and cancer (Yamada et al., 2015). This evidence concerns the gene CC2D1A and cancer.