CDG linked to ichthyosis or ichthyosiform dry skin with variable neurologic and multi-organ involvement are due to deficiencies within the dolichol (DOLK-, SRD5A3-CDG) and the GPI (glycosylphosphatidylinositol) anchor biosynthesis pathway (PIGL-CDG, MPDU1-CDG) (Schenk et al., 2001; Kranz et al., 2007; Kahrizi et al., 2011; Lefeber et al., 2011; Kapusta et al., 2013; Jaeken et al., 2014; Rymen and Jaeken, 2014; Rush et al., 2017; Thiel et al., 2018; Hall et al., 2020; Ng et al., 2021). This evidence concerns the gene DOLK and ichthyosis.