A 5-year-old girl had a 2-week history of protruding bilateral eye with blurred vision. The patient had a history of low-grade fever. Bilateral proptosis with a markedly severe protrusion of the conjunctival palpebral and infraorbital tissues was observed. A more pronounced proptosis occurred on the left eye. Lagophthalmos and corneal defects were found in the left eye. Laboratory tests revealed anemia, hyperleukocytosis, and thrombocytopenia. A confirmed diagnosis of acute myeloid leukemia-M2 was established from bone marrow aspiration. The patient was treated with standard multiagent chemotherapy and topical antibiotic eyedrops. Proptosis had resolved in 4 months with residual corneal cicatrix in the left eye.