GATA2 deficiency is a complex and heterogeneous multisystem disorder with a broad spectrum of clinical and morphologic manifestations, encompassing conditions with overlapping characteristics such as Emberger syndrome, monocytopenia, and mycobacterial infections syndrome, or immune deficiency marked by monocytopenia, reduced dendritic cells, natural killer cells, and B lymphocytes [1,2,3,4]. The gene discussed is GATA2; the disease is hyperinsulinemic hypoglycemia, familial, 4.