A 13-year-old female child presented with bilateral proptosis and subconjuctival hemorrhage of 2 months duration. Subsequently, the biopsy and peripheral smears confirmed the diagnosis of acute myeloid leukemia (AML). Recurrent cytogenetic translocation t(8:21) in AML associated with extramedullary manifestation; was discovered which is a rare event. Furthermore, myeloperoxidase negative blasts and periodic acid Schiff negative blasts in AML with t(8:21) is a very rare combination to the best of our knowledge.