Essential thrombocythemia is a myeloproliferative disorder characterized by an elevated platelet count, with symptoms such as vasospasm and a predisposition to bleeding and microvascular complications. While rare, one complication that can arise from chronic portal vein thrombosis is the cavernous transformation of the portal vein. In this case report, we explore a unique presentation of cavernous transformation of the portal vein (CTPV) in a patient diagnosed with essential thrombocythemia (ETC). This case report gives importance to considering myeloproliferative disorders like ETC as a potential cause of chronic portal vein thrombosis leading to the CTPV.This case report describes a 26-year-old male patient diagnosed with cavernous transformation of the portal vein (CTPV) during a workup for hematemesis and melena. The patient was found to have severe anemia, thrombocytosis, splenomegaly, and a positive JAK2 mutation, consistent with a diagnosis of essential thrombocythemia. Although thrombotic events in essential thrombocythemia patients are common, portal vein thrombosis and CTPV are rare complications. Cavernous transformation of the Portal vein is a condition that is seen in the setting of long standing portal vein thrombosis. There are diverse causes to be considered when chronic portal vein thrombosis leading to cavernous transformation is encountered clinically and radiologically. In this case report the predisposing condition was found to be essential thrombocythemia. The article emphasizes the importance of considering cavernous transformation of the portal vein (CTPV) as a complication in patients with essential thrombocythemia. Keywords: Essential thrombocythemia, Portal vein thrombosis, myeloproliferative neoplasms, Cavernous transformation of the portal vein, case report.