The current cohort of IEI patients with non-hematologic cancers and solved molecular diagnosis showed defects in DNA repair pathway (ATM deficiency), T cell development (AIRE deficiencies and STAT1 gain-of-function), B cell development (IKZF1 and PI3KR1 deficiencies), and innate immune pathway (IL12RB1 deficiency). This evidence concerns the gene IKZF1 and hematopoietic and lymphoid cell neoplasm.