Abstract Introduction We present a case of a 9‐year‐old girl diagnosed with a myeloproliferative neoplasm (MPN) harboring both monosomy 7 and an ALK/ROS1 fusion gene. Case presentation The neoplasm was resistant to conventional AML chemotherapy and required hematopoietic cell transplantation (HCT) to achieve remission. Discussion MPNs with monosomy 7 and ALK/ROS1 fusions occur in a wide age range of children and adults, and require HCT for long‐term remission. Furthermore, these cases can be responsive to ALK inhibitors. Conclusion This report underscores the potential need to reclassify such MPNs as a distinct entity, which has unique therapeutic implications.