Essential thrombocythemia is a myeloproliferative neoplasm characterized by platelet aggregation and thrombosis. Clinically, essential thrombocythemia increases the risk of both thrombosis and bleeding. Essential thrombocythemia is more involved in micro- and small-sized arteries than in large arteries. Many essential thrombocythemia patients exhibit various symptoms, including microvascular thrombosis with acute coronary disease, digital ischemia, and transient ischemic attack. This study reports a rare case of recurrent thrombosis in relatively large vessels including splanchnic, lower extremity arteries, and aorta in essential thrombocythemia. A 70-year-old woman was admitted to the emergency room with abdominal pain and fever for a day. The patient underwent three operations due to recurrent arterial thrombosis of superior mesenteric, splenic, aorta, and lower extremities. She had recurrent diarrhea and acute kidney injury because of short bowel syndrome after extensive bowel resection. In conclusion, essential thrombocythemia patients aged >60 years and who have risk factors such as history of major ischemic events or severe leukocytosis must be careful of thrombosis of the medium- and large-sized arteries, including splanchnic and lower extremity arteries.