What is behind umbilical appendix in neonates that we need to know?

Anomalies of the vermiform appendix involving the umbilicus are exceptionally uncommon. The few cases described in the literature reveal a broad clinical spectrum, highlight diagnostic challenges, and provide important insights into embryological mechanisms. We report a 7-day-old female presented with a flashy tubular structure from the umbilicus, there was a dark-red structure about 3.5 cm in length and having a diameter of about 3 mm. Exploration of the umbilicus with incision extended to the right transverse of the abdomen revealed an external tubular structure communicating with the cecum as the vermiform appendix. Umbilical appendix, though rare, presents important diagnostic and surgical challenges. They may mimic common umbilical conditions. Careful inspection of the umbilicus in neonates and precise use of terminology can further improve diagnosis, prevent iatrogenic injury, and guide appropriate management.