Pyloric atresia (PA) is rare and may be associated with epidermolysis bulosa (EB). No case of PA.treated by laparoscopic surgery has been reported. The authors report the first case of neonate with PA and ES, treated successfully by laparoscopic surgery. That is a full term girl, birth weight 2000g, admitted to our hospital with respiratory distress, non-bilious vomiting, lots of blister and erosions on the skin. Plain abdominal X-ray showed one big air bubble of the stomach. Upper gastrointestinal contrast study confirmed complete obstruction of the pylorus. The child was operated at the age of 7 days by laparoscopic approach, using 3 trocars 3-5mm. Complete pyloric obstruction by a thick membrane 5mm was found intra-operatively. Resection of the membrane and pyloroplasty by Heneke-Mickulicz were carried out. The child recovered uneventfully after the operation, started oral feeding at postoperative day 7 and was discharged 5 days later.