Tension gastrothorax in children is a life-threatening condition and presents dramatically with acute and severe respiratory distress. It develops when an intra-thoracic stomach herniated through a diaphragmatic defect is massively distended by trapped air and/or fluid causing mediastinal displacement. Tension gastrothorax is often misinterpreted as tension pneumothorax and managed as such leading to increased morbidity and mortality. We present a child with tension gastrothorax and a literature review of this phenomenon.Immediate clinical and radiographic evaluation should lead to accurate diagnosis followed by emergency decompression of the stomach before laparotomy with reduction of herniated viscera and repair of the diaphragmatic defect.