Homozygous or compound heterozygous pathogenic variants in ATM gene are associated with Ataxia-Telangiectasia (MIM #08900), a multisystemic disorder with neurological and immunological involvement, and marked susceptibility to hematological and solid malignancies with autosomal recessive inheritance, including OC [7, 8]. The gene discussed is ATM; the disease is ataxia telangiectasia.