Mediastinal cysts in neonates are infrequently reported. Enteric cysts in the posterior mediastinum are mostly asymptomatic. Vertebral malformations may contribute. Clinical presentation may include respiratory distress. Herein, we report a case of mediastinal gastroenteric cyst and meningomyelocele in a neonate presented respiratory distress signs after delivery. The patient was well managed by excision of mediastinal cyst and closure of dura mater of meningomyelocele. A detailed antenatal scan may help in early diagnosis and better outcome. Moreover, vertebral anomaly in chest radiograph of infants with respiratory distress should suggest the possible presence of a mediastinal enteric cyst
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