Primary volvulus of the small intestine is rare in neonates. We report a case of primary volvulus of the ileum in a preterm newborn. Vomiting and abdominal distension began 10 hours after birth, followed by hypovolemia, hyponatremia, and acidosis. Plain abdominal radiograph revealed a pattern of diffuse ileus and very dilated loops at the right upper quadrant of the abdomen. Three days later, another plain film showed increased fluid accumulation and decreased air in these dilated loops. Emergency laparotomy revealed primary volvulus of the distal ileum with gangrenous change. Resection of the necrotic ileum with an end-to-end ileoileostomy was performed and the patient recovered fully. In a newborn with ileus and rapidly deteriorating hemodynamics, early surgical intervention is essential to achieve good prognosis.