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Inguinal hernia with acute appendicitis known as Amyand's hernia is uncommon. It occurs mostly in adults, older children and infants. The appendix inside the hernial sac is rarely perforated. The authors describe in a neonate aged 14 days an unusual case of inguinal hernia containing a perforated appendix that was clinically considered as a strangulated inguinal hernia. Surgery performed with an inguinal approach permitted us to remove the appendix and close the hernial sac. The postoperative course was uneventful after a follow-up of 1 year.