We report on an infant with neonatal Marfan syndrome (NMS) and hiatus/paraesophageal hernia who presented to a university hospital with an unusual early complication of this connective tissue disorder. An abnormal course of the nasogastric tube was noted on the first day of life by a radiograph of the chest and abdomen performed for bloody gastric drainage. The question of esophageal perforation was raised. Subsequent contrast study demonstrated a large hiatus/paraesophageal hernia with pronounced gastroesophageal reflux (GER). A part of the hernia was positioned posterior and to the right of the gastroesophageal junction (GEJ), presumably the location of the nasogastric tube as noted on the initial films. Although characterized by cardiac/aortic abnormalities, NMS can be a difficult diagnosis and should be considered in any infant with hiatus/ paraesophageal hernia with or without GER.