Rapunzel syndrome is very extreme form of trichobezoar formation where the tail of the trichobezoar extends from the stomach into the small intestine. Death resulting from this condition is rare and is usually associated with gastric or intestinal perforation. We report a fatal case of Rapunzel syndrome in a 3 years and 10 months old girl. Review of the literature indicates that this case involves the youngest child to have died from this syndrome. Furthermore, this case is unique due to the clear association with the parent's neglect with failure to provide the child with adequate health care.