To study the clinical and pathological characteristics of myeloid sarcoma (MS), we collected 1 case of childhood orbital leukocyte MS and 1 case of adult orbital solitary MS, and analyzed the clinicopathologic features and reviewed literature. Both patients are male, aged 9 years old and 36 years old. They were admitted to the hospital with symptoms such as prominent protrusions of the eyeball, eye movement disorders, and eyelid edema. Tumor cells were diffuse under the light microscope of the tumor resection specimen. The morphology was relatively consistent, medium or large, cytoplasm was small and light stained. The nucleus was round, oval or irregular, some cells had vacuoles, nucleolus was obvious, and nuclear division was easy to be seen. It can be seen that scattered in the distribution of eosinophilic granulocytes. The tumor cells infiltrated into around tissues; two cases of immunohistochemistry were positive for myeloperoxidase (MPO), lysozyme, CD117, CD34, CD31, and CD43. The eye symptoms of two patients after surgical resection were significantly relieved. The 9-year-old patient was diagnosed as acute myeloid leukemia (AML) after external bone marrow smear. AML1/ETO fusion gene was detected by FISH and was eventually diagnosed as left orbital Leukocyte MS. Another patient was followed up for 2 years without evidence of leukemia and was diagnosed as isolated MS in the right orbital orbit. The orbital MS is rare and lacks characteristic clinical manifestations. It is treated only with orbital symptoms and is often misdiagnosed and untreated, in particular, the leukocyte MS and orbital solitary MS as the first symptom. It should be combined with immunohistochemistry, molecular detection, and bone marrow detection to distinguish it from other tumors.