DOCK8 is an atypical guanine nucleotide exchange factor of the DOCK 180 superfamily, which interact with Rho GTPases regulating cytoskeletal rearrangements [1, 5, 10, 11], partially explaining phenotypical overlap with other IEIs caused by cytoskeletal dysregulation, especially Wiskott-Aldrich syndrome (WAS) and CARMIL2 deficiency [12, 13]. The gene discussed is DOCK8; the disease is Wiskott-Aldrich syndrome.