Agnogenic myeloid metaplasia, associated with myelofibrosis, is a myeloproliferative disorder. Extramedullary hematopoiesis in the pleura is rare and its prognosis is often severe.Herein we report a 64-year-old woman, who presented with pleural extramedullary hematopoiesis, treated by hydroxyurea-based chemotherapy with disease control.Clinical, histological, therapeutic and evolutive aspects of this uncommon entity will be reviewed.