Monochorionic-monoamniotic (MCMA) twin pregnancy discordant for anencephaly is a rare occurrence. Management of such cases using Selective reduction versus expectant management is itself a questionable topic. We report such a case managed expectantly with successful outcome. A 26 year old primigravida woman was referred to us with MCMA twin pregnancy discordant for anencephaly at 21+2 weeks of gestation. She opted for expectant management till 34 weeks and elective cesarean section was performed. Healthy neonate without any congenital malformation was 2.1Kg and required 4 days of NICU care. Neonate with anencephaly weighing 1.4Kg succumbed after 22 hours of life. This report emphasizes the option of expectant management for such a rare condition.

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