ZMYM2 is a zinc finger transcriptional corepressor that plays critical roles in developmental gene silencing and transposable element control. It functions as part of distinct chr13-associated complexes, including interactions with LSD1, HDAC1, ADNP, and TRIM28, through which it represses germline genes and different classes of retrotransposons 1. During early embryogenesis, ZMYM2 is essential for DNA methylation and silencing of germline gene promoters and active LINE elements; homozygous loss results in embryonic lethality by E10.5 2. Heterozygous loss-of-function mutations cause neurodevelopmental-craniofacial syndrome with variable renal and cardiac abnormalities (NECRC), characterized primarily by congenital anomalies of the kidney and urinary tract (CAKUT) but also manifesting with neurodevelopmental features, craniofacial dysmorphisms, and spastic motor signs 34. In cancer, ZMYM2 participates in oncogenic fusion events, most notably ZMYM2-FGFR1 fusions associated with myeloproliferative neoplasms and the ZMYM2-FLT3 fusion in myeloid/lymphoid neoplasms with eosinophilia, both of which drive IL-3-independent proliferation and chr13 instability 56. Tyrosine kinase inhibitors effectively suppress ZMYM2-FLT3-driven proliferation and promote apoptosis 6, offering a therapeutic approach for fusion-positive neoplasms.