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A 10-year-old girl presented with a 2-week history of an elevated, red conjunctival mass. She was in remission from acute myeloid leukemia (AML) and had two recurrences. Biopsy revealed a conjunctival myeloid sarcoma, an extramedullary lesion composed of cells of myeloid lineage that may present in children and adults, most commonly in the lymph nodes, skin, and subcutaneous tissues; it is less likely in ocular tissue. In patients with a history of AML, a new myeloid sarcoma is considered a relapse. To the best of our knowledge, this is the first case of a conjunctival myeloid sarcoma heralding a recurrence of an aggressive form of AML in a child.