Background: Primary myelofibrosis is a rare myeloproliferative disorder that is occasionally associated with abdominal and hepatic complications, such as Budd–Chiari syndrome, nodular regenerative hyperplasia, portal vein thrombosis, and rarely portal hypertension, which is found in 7% of the cases. Case Presentation: We report a rare case of portal hypertension with chylous ascites in a 49-year-old male patient with primary myelofibrosis, who presented with a painless progressive distension of the abdomen for 1 month. His transjugular liver biopsy revealed extramedullary hematopoiesis with colonies of erythroid precursors, megakaryocytes, and numerous clusters of erythroid islands in the hepatic sinusoids. He was provided with a salt-restricted diet and diuretics with partial response and was scheduled for a transjugular intrahepatic portosystemic shunt. Conclusion: Extramedullary hematopoiesis should always be considered in patients with myeloproliferative disorders with a rare case of portal hypertension.

Keywords: Myeloproliferative disorders, portal hypertension, chylous ascites, extramedullary hematopoiesis, primary myelofibrosis, case report