Neonatal terminal ileum volvulus in the absence of malrotation has never been reported before in English literature. However, another similar rare entity known as neonatal primary segmental volvulus without malrotation has been reported before. Volvulus, in general, is an extreme emergency and cases not diagnosed in time lead to death. The main diagnosis is based on radiological features seen on imaging. We present a case of volvulus of terminal ileum that was diagnosed and surgically treated at age of 15 h ensuring the newborn survived. The definitive diagnosis was based mainly on ultrasonographic findings.