Enterobius vermicularis is one of the most common intestinal parasites found in humans. They commonly infest the terminal ileum and large intestine, and are usually considered an innocuous parasite that can be easily eradicated with proper treatment. However, extraintestinal migration of worms, although very rare, may lead to severe health disorders or even death. This article, reports the first fatal case of ectopic enterobiasis known to the authors, which developed in an adult patient with E. vermicularis infection, causing perforation of the large intestine and generalized bacterial peritonitis. Despite emergency laparotomy, the patient died from septic shock on the day after surgery. During pathological examination, worms were found not only in the large intestine, but also in the renal parenchyma; worm eggs were found deposited in the lungs as well.