A 39 year old Caucasian female with a past medical history of vWD  and melanoma s/p resection 15 years ago presented with abdominal distension and discomfort for several months. Physical examination revealed an enlarged spleen palpable in the left lower quadrant. Initial labs revealed pancytopenia with a white cell count of 3.60, hemoglobin of 8.8 and a platelet count of 90,000. Peripheral blood smear revealed dacrocytes and nucleated red blood cells. CT abdomen confirmed the presence of massive splenomegaly (Panel A, arrows) along with skeletal sclerosis suggesting extra medullary hematopoiesis. A bone marrow aspirate resulted in a ‘dry’ tap and a core biopsy was obtained. The biopsy showed a hypo-cellular marrow (<5%), prominent stromal fibrosis and marked osteosclerosis (Panel B, arrows) consistent with fibrotic phases of primary myelofibrosis. The patient will start Ruxolitinib and will also be referred for Bone Marrow Transplant evaluation.