<p>Myelofibrosis (MF) is a&#xa0;rare clonal disease of the haematopoietic stem cell characterised by pathological activation of the JAK/STAT signalling pathway. The disease typically manifests clinically as anaemia, splenomegaly and constitutional symptoms. The therapeutic decision between watchful waiting and active treatment is based primarily on the risk profile, symptoms and molecular genetic findings. While low-risk patients without symptoms can be monitored, those at higher risk or with significant symptoms benefit from early treatment, especially with Janus kinase (JAK) inhibitors. Ruxolitinib has been the standard of care since 2012, and newer substances such as momelotinib are expanding the spectrum, especially in anaemic patients. For suitable patients, allogeneic stem cell transplantation remains the only curative option. Future developments are aimed at personalised, disease-modifying therapy that goes beyond mere symptom control.</p>