Primary esophagoesophagostomy is the treatment of choice for repair of esophageal atresia, particularly the more common type C atresia. Debate continues, however, regarding repair of the type A, or long-gap esophageal atresia. Since the pioneering work of Livaditis, Howard and Myers, the frequency of primary repair of long-gap atresia has increased. Interposition grafting, however, remains as the treatment for gaps longer than 6 cm or with absence of a distal intrathoracic esophageal segment. We report a case of primary esophagoesophagostomy with proximal esophagomyotomy in a 1,900-g infant with an absent distal intrathoracic esophageal segment and an eight vertebral body gap. Utilizing daily bougienage of the proximal and eventually the distal segment, a 1-cm gap was present at 6 months of age. With intraoperative fluoroscopy, the distal intra-abdominal esophageal segment was manipulated thru the esophageal hiatus. Thru an extrapleural approach with a proximal esophagomyotomy, a delayed primary anastomosis was successfully performed. She is currently 3 years old and has a normal barium swallow without stricture or gastroesophageal reflux.