Thirty four neonates presented with acute duodenal obstruction due to malrotation during a 9 year period between 1973 and the end of 1981. Of these, 20 patients (58.8%) presented in the first week of life, and 24 (70.5%) had an associated midgut volvulus. This frequent association is stressed as bowel necrosis occurs very rapidly. Massive gangrene of small bowel was present in 5 patients, extensive resection was necessary in 3 patients, of whom 2 died postoperatively (5.8%). Five patients required reoperation and 10 had additional G.I. malformations (29.4%).