Type II paraesophageal hiatal hernia is a rare entity that is rarely discussed in the literature. This report is intended to depict the clinical profile of the disease and to discuss several controversial issues involved in the repair, including indications for surgery, the most appropriate surgical approach, and the need for a concomitant antireflux procedure. This study retrospectively reviews the experience with 12 patients affected by paraesophageal hernia who underwent a surgical repair between 1973 and 2001. Ten were women and two were men, with a mean age of 56.8 years. Clinical features and diagnostic assessment, as well as operative findings, are presented. Nine patients underwent an elective operation, and three patients underwent an emergency procedure for hernia complications. A thoracic approach was used in one patient, whereas the remaining 11 patients underwent an abdominal repair; the hernia sac was resected, and the hiatus was reconstructed in all of the patients. No postoperative deaths occurred; complications occurred in one patient, and only one recurrence was observed. Type 11 paraesophageal hernia seems to be a rare primary diaphragmatic defect. Its surgical repair is mandatory, because paraesophageal hernia is a potentially life-threatening disease because of the risk of severe complications; an elective repair should be performed wherever possible, even in asymptomatic patients. The main problems of the management are the choice of the surgical approach and the usefulness of an associated antireflux procedure to the hiatoplasty; in our opinion, a fundoplication should be added to all repairs.