Duplex Appendix vermiform is rare entity and constitutes a total of 0.004 % to 0.009% documented incidence. Duplex appendix is mostly found incidentally during laprotomy for some other causes and sometimes inflammation of an appendix leads to the diagnosis. Here we present 11 days old baby girl with presumptive diagnosis of ano-rectal malformation with grossly distended abdomen, meconuria and absent anal opening, although baby was passing stool from single anterior opening. Her X-ray abdomen shows distended bowel with ground glass appearance at lower abdomen. On exploration for colostomy, we found congenital pouch colon with duplex appendix. To our knowledge this is a unique case of duplex appendix with congenital pouch colon and ano-rectal Malformation.
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