TheMN1::ETV6gene fusion resulting from t(12;22)(p13;q12) has been rarely reported in myeloid neoplasms. We describe a 69-year-old male with newly diagnosed acute myeloid leukemia (AML) with erythroid differentiation and t(12;22)(p13;q12) demonstrated by conventional chromosome studies. Subsequent fluorescencein situhybridization studies demonstrated a balancedETV6gene rearrangement (at 12p13). To further characterize this translocation, whole-genome sequencing was performed which confirmed t(12;22) with breakpoints involving theMN1andETV6genes. Herein, we describe our case and review the literature to summarize the clinical and laboratory findings in patients with this rare but recurrentMN1::ETV6gene fusion observed in myeloid neoplasms. Importantly, this case expands the clinical spectrum associated with theMN1::ETV6gene fusion to include AML with erythroid differentiation. Lastly, this case demonstrates the importance of moving toward more comprehensive molecular testing to fully characterize the driver events in neoplastic genomes.