Primary myeloid sarcoma occurring in multiple sites; orbit, ear, brain, and spinal cord is a rare clinical entity. A 15-year-old male adolescent presented with bilateral orbital mass, hearing difficulty, and clinical signs of multiple cranial nerves palsy. Approximately 6 weeks later, acute myeloid leukemia was confirmed. This case alerts us that in patients with diverse sarcomatous lesions, acute myeloid leukemia presenting as myeloid sarcoma should be considered.

Keywords: acute myeloid leukemia, myeloid sarcoma, t(8;21)