| Summary: | Introduction: Neonates presenting with bilious vomiting require emergent evaluation to rule out malrotation with volvulus. Enteric duplication cysts are congenital gastrointestinal lesions that can cause intestinal obstruction. However, they are not typically associated with a presentation and imaging findings suspicious for intestinal malrotation with volvulus. Case presentation: An eight-week-old male presented with progressive feeding intolerance, weight-loss, and bilious vomiting. Upper gastrointestinal series showed a complete obstruction at the ligament of Treitz with a “bird beak” sign. Due to the clinical assessment and imaging findings, malrotation with volvulus was suspected and the patient underwent surgical exploration during which an obstructing enteric duplication cyst was found on the proximal jejunum. The duplication cyst and the adjacent jejunum were resected, and bowel continuity was restored by a primary anastomosis. The diagnosis of an enteric duplication cyst was confirmed on the histopathological examination. Conclusion: To our knowledge this is the first reported case of a jejunal enteric duplication cyst located at the ligament of Treitz leading to intestinal obstruction and having findings on imaging studies mimicking those of an intestinal malrotation with volvulus. Enteric duplication cysts should be included in the differential diagnosis of newborns with acute bilious vomiting.
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