Congenital absence of the appendix in a child with malrotation

Congenital absence of the appendix is an extremely rare condition. Often found incidentally during surgical procedures, it poses a surgical dilemma. Surgeons must be well versed in the congenital abnormalities and positional variations of the appendix and must know the steps required during surgery to diagnose this abnormality. In this case report, we discuss the first ever occurrence of congenital absence of appendix in association with malrotation.