Purpose: The aim of this work is to discuss the pathogenesis of the segmental dilatation of the intestine (SDI) and to revie wits clinical presentation and the ways to confirm the diagnosis.Methods: Eight cases of pathologically proven SDI from 1987 to 2003 were reviewed and discussed. There were 7 newborns and a 1-year-old boy. Results: Our patients are 5 boys and 3 girls. In all cases, the diagnosis was not suspected before surgery. Two patients presented with a low neonatal bowel obstruction. Six patients were operated for omphalocele,which was the most frequent associated malformation.The SDI involved the ileum in all patients. The treatment consisted on a resection of the dilated segment with an end-to end anastomosis. Histological examination demonstrated the presence of ganglion cells in all cases. The muscular layer was hypertrophied in two cases and very thin in one case. A heterotopic gastric mucosa was observed in one case.No anomalies were observed in 5 cases. The postoperative course was uneventful in 6 cases with a mean follow-up of 5 years. Conclusions: Segmental intestinal dilatation is an exceptional pathology with an unknown etiology and a misleading clinical presentation. Several theories were proposed to explain this malformation; however, most authors are rather inclined to an embryological theory incriminating an extrinsic intrauterine intestinal compression. Most cases are neonatal discoveries. The clinical polymorphism and the lack of specificity of radiological investigations explain the difficulties to have a preoperative diagnosis. However, this difficulty is compensated by the favorable evolution after the resection of the dilated segment. Purpose: The aim of this work is to discuss the pathogenesis of the segmental dilatation of the intestine (SDI) and to revie wits clinical presentation and the ways to confirm the diagnosis. Methods: Eight cases of pathologically proven SDI from 1987 to 2003 were reviewed. There were 7 newborns and a 1-year-old boy. Results: Our patients are 5 boys and 3 girls. In all cases, the diagnosis was not suspected before surgery. Two patients presented with a low neonatal bowel obstruction. Six patients were operated for omphalocele, which was the most frequent associated malformation. The SDI involved the ileum in all patients. The treatment consisted on a resection of the dilated segment with an end-to end anastomosis. Histological examination demonstrated the presence of ganglion cells in all cases. The muscular layer was hypertrophied in two cases and very thin in one case. A heterotopic gastric mucosa was observed in one case. No anomalies were observed in 5 cases. The postopera tive course was uneventful in 6 cases with a mean follow-up of 5 years. Conclusions: Segmental intestinal dilatation is an exceptional pathology with an unknown etiology and a misleading clinical presentation. Several theories were proposed to explain this malformation; however, most authors are而且, 这个 困难 是 compensated by the favorable evolution after. However, this difficulty is compensated by the favorable evolution after the resection of the dilated segment.