Patients with essential thrombocythemia (ET) can experience hemorrhagic or ischemic vascular events. The prevention of these complications is challenging, and the overall risk of vascular events caused by ET is often overlooked. A 34-year-old man was admitted for a 10-day history of weakness and numbness in his right limbs. He had been diagnosed with ET in 2008 but had stopped receiving treatment half a year before admission. Physical examination showed a superficial sense of disturbance in the right limbs and decreased muscle strength in the right upper and lower limbs (4/5). His platelet count (459 × 10/L) was elevated. Magnetic resonance imaging showed acute watershed infarction, and he was treated successfully. However, he was readmitted for headache and left limb weakness 14 months later. A head computed tomography scan revealed spontaneous subdural hemorrhage. He underwent subdural hematoma removal and decompressive craniectomy. Surgery and pathological investigation revealed no venous sinus thrombosis or vascular malformation. His condition improved, and he exhibited a stable condition 1 year after discharge. Successive development of ischemic stroke and spontaneous subdural hemorrhage is rare in a patient with ET. This case suggests that ET is not only a risk factor for stroke but can also cause highly heterogeneous strokes.