Primary segmental volvulus of the small intestine is not associated with malrotation, malfixation of the midgut, nor other primary small bowel lesions such as small bowel tumors. This entity is known to be more prevalent in adult and in certain global areas associated with particular diet habits. There have been very few reports in neonates, but not in this country so far. The author reports two cases of primary segmental volvulus. Case 1 was a septic 4-day-old girl with hematochezia due to jejunal volvulus with partial necrosis and panperitonitis. Resection of the segment and Bishop-Koop enterostomy were successful. Case 2 was a 3-day-old boy, who had ileal volvulus with ultra-short length of ileal atresia, probably due to intrauterine segmental volvulus. Limited resection of the atresia and spreading of the mesenteric base were enough to recovery. The rarity of the pathognomonic findings and limitation of the diagnostic workup due to rapid progression limit early diagnosis and good survival rate in this particular condition.