Clinical observations of sporadic cases of MIA confined to the small bowel lend support to the hypothesis of a vascular incident etiology. One-stage restoration of intestinal continuity with preservation of maximal intestinal length should be the basic principle of any operative management of MIA. Despite a relatively high morbidity related to the primary damage of the fetal intestine, excellent results with 100 % survival rates can be obtained. After taking the differences in pathogenesis, anatomical and histological features, and the prognosis for sporadic and hereditary forms of MIA into account, these two entities should be classified separately in a modified classification of intestinal atresia.