During a 13-month period, 13 patients with asplenia syndrome were evaluated with MRI for cardiovascular and visceral anomalies. The MR images were reviewed for the presence of haitus hernia which was found in three patients. One of the remaining ten patients with no MRI evidence of hiatus hernia was diagnosed as having gastro-oesophageal reflux and hiatus hernia by an oesophagogram and 24-h pH monitoring. This patient had undergone fundoplication prior to MRI. Out of the 13 patients (31%) with asplenia syndrome, 4 had hiatus hernia. It appears that among patients with the asplenia syndrome, hiatus hernia is a frequent finding. Recurrent pneumonia or bronchiolitis in patients with asplenia syndrome requires evaluation for the presence of hiatus hernia and gastro-oesophageal reflux.