A 29-year-old woman, gravida 1, admitted to the institutional Maternal-Fetal Medicine Unit at 36 weeks of gestation, with previous normal ultrasound studies. The ultrasound finding reported absent fetal stomach, no mediastinal deviation, no ultrasound signs of swallowing alterations and normal values of amniotic fluid volume. Ultrasound monitoring was realised every 72 hours during the first week of evaluation and weekly until the end of pregnancy, and the same ultrasound findings persisted. Her family history only included a family history of a cousin with esophageal stenosis. At 39 weeks of gestation, a male newborn weighing 3145 g was delivered by elective Caesarean section, with Apgar scores of 9 and 10 at 1st and 5th min respectively. During the newborn adaptation, a nasogastric tube was inserted with subsequent abdomen and chest X-ray control that showed dilatation of the retrocardiac portion of the esophagus and the tube rolled up inside it. A contrast pharyngography and esophagram reported ascension of the stomach to the thorax with volvulus, filiform passage of contrast medium to the duodenum and episodes of gastroesophageal reflux without the presence of tracheoesophageal fistula. At 24 hours of life, surgical correction of posterior paraesophageal hiatal hernia by laparotomy was performed without complications, with radiographic control after the procedure, clinical follow-up of the newborn and indication to restart the enteral feeding in 48 hours with adequate tolerance. On the eighth day of life, the newborn was discharged from hospital. One month after the procedure, the newborn is growing adequately and without complications. We propose that in the absence of abnormalities of the lower airway and normal values of amniotic fluid in the third trimester; the isolated ultrasonographic finding of absence of fetal stomach could be suggestive of congenital hiatal hernia.