An 82-year-old woman who had a history of essential thrombocythemia presented with ocular pain, bleeding, and decreased visual acuity of the left eye. Orbital computed tomography revealed a relatively well-defined homogenous mass-like lesion in the left subconjunctival and intraconal space. Conjunctival biopsy showed acute inflammation with necrosis, vascular ectasia with thrombosis and hemorrhage. After the treatment with hydroxyurea and anagrelide, her symptom and lesion were markedly improved. Hematologic disorders like essential thrombocythemia should be considered in patients with severe spontaneous bleeding around the eye.