MDS with monosomy 7 frequently occurs in patients with germline variants in GATA-binding factor 2 (GATA2), sterile alpha motif domain containing 9 (SAMD9), sterile alpha motif domain containing 9 like (SAMD9L), or hereditary bone marrow failure syndrome [12]. The gene discussed is SAMD9L; the disease is myelodysplastic syndrome.