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Hemoglobin O-Arab [Beta 121 Glu → Lys] (Hb O-Arab) is a rare abnormal hemoglobin (Hb) whose clinical and hematological features have been described in adults but not in children. We report three children, 9, 12, and 36 months of age, with homozygous Hb O-Arab and assess the value of supplementary folic acid as treatment. Pediatr Blood Cancer 2013; 60: 506–507. © 2012 Wiley Periodicals, Inc.