Aortic thrombus is rare in patients with essential thrombocytosis (ET), so the optimal treatment remains undefined. A 45-year-old man with history of ET, under chronic treatment with aspirin, presented to the emergency department complaining of acute onset in both the legs and abdominal pain. Physical examination revealed that both dorsalis pedis pulses were not palpable with cold and pale feet. His abdomen was soft and nondistended. The platelet count was 436 x 10(9)/L. The thoraco-abdominal computerized tomographic scanning revealed normal aortic diameter with supraceliac and infrarenal nonoccluding thrombus and infarction areas in spleen and left kidney. At the emergency department he presented with recurrent symptoms, losing bilateral posterior tibial pulses. A decision was made to perform a thoracoretroperitoneal incision. A longitudinal sequential aortotomy was performed in the distal thoracic and infrarenal aorta, and the thrombus was easily removed. Following this, he underwent bilateral crural thrombectomy and local intra-arterial thrombolytic therapy. The postoperative course was uneventful. The left toes were amputated because of necrosis. He was discharged and put on antiaggregants, anticoagulants and hydroxyurea. Aortic thrombus in patients with ET is unusual, but potentially lethal. There is complete relief from symptoms in recurrent cases following surgery. An appropriate medical treatment after intervention must be supported.