Primary myelofibrosis is a form of myeloproliferative neoplasm characterized by stem-cell derived clonal proliferation, resulting in bone marrow fibrosis, splenomegaly and extramedullary haematopoiesis, with portal hypertension (PHT) occuring in 10%–20% of patients. Here, we report a patient with primary myelofibrosis whose PHT improved significantly following treatment with ruxolitinib, thereby allowing discontinuation of all drug therapy for PHT.