Aim: In this study a case of wind sock anomaly is presented due to its rare localization.

Case: A 10 days old girl with Down syndrome was brought to our unit with the complaint of bilious vomiting. Triple air-fluid levels in the epigastric region and scarce gaseous images in the distal intestinal system were detected in the upright abdominal X-ray. The patient was operated with the diagnosis of proximal intestinal obstruction. Perforated wind sock anomaly was detected in the proximal jejunum. Membrane ablation and primary repair was done. Pathological examination revealed jejunal mucosa in the membrane.

Conclusion: Wind sock anomaly in the proximal jejunum was not reported in the literature before. The probability that this types of anomalies can be detected in the gastrointestinal system other than duodenum is emphasized.