Our study set included patients with both high and moderate penetrance solid cancer risk variants in DNA repair genes, e.g., TP53, BRCA1, BRCA2, CHEK2, and FANCM. To improve long-term survival, these patients require individualized cancer surveillance plan as part of the HSCT follow-up, and in some cases tailoring of the conditioning modalities. The gene discussed is CHEK2; the disease is cancer.