A 51-year-old female with esophageal stricture was referred to our hospital. She was diagnosed to have mixed connective tissue disease and had been placed on steroid and immunosuppressant treatment. She presented with passage disturbance and free reflux of the gastric contents when in the supine position. Pneumatic dilatation and medication resulted in partial relief of her symptoms. Preoperative imaging studies demonstrated a shortened esophagus with severe stricture of the esophagogastric junction and a moderate hiatal hernia. A DeMeester's score of 140.1 was noted on 24-h pH monitoring. Under a diagnosis of stricturing reflux esophagitis, surgical treatment was indicated. Laparoscopic transhiatal mediastinal dissection with crural repair and fundoplication was offered instead of thoracotomy and/or laparotomy, since she had a high risk due to immunosuppression. The esophagus was extensively dissected through the hiatus up to the level of the tracheal bifurcation, and fundoplication was completed without Collis gastroplasty. Her postoperative course was rapid and uneventful. Postoperatively, her clinical symptoms were resolved with anatomical/functional improvement.