Acute appendicitis is one of the most common surgical emergencies in paediatrics. However, acute appendicitis in early infancy is an uncommon condition. Furthermore, strangulation of the small intestine through appendicular knotting is described as very unusual in the literature and is generally not well-diagnosed in the clinical context. This article reports the case of a 23-month-old girl who entered the emergency department with a three-day history of abdominal symptoms and who died in less than 24 hours without receiving surgical intervention. The case turned judicial at the request of the parents who claimed lack of clarity in the diagnosis. A medico-legal autopsy was ordered to clarify the cause and manner of death. The autopsy documented herniation, strangulation and torsion of a 70 cm segment of the jejunum/ileum through an appendicular knot caused by the attachment of the distal end of the inflamed appendage to the ileum. The case is relevant because it is the first case of death by appendicular knot and strangulation of small bowel in an infant reported in the literature. The importance of autopsy to clarify the clinical diagnosis is noted.