Management of esophageal atresia with tracheoesophageal fistula (TEF) has evolved over the past 5 years at Children's Hospital and Medical Center, Seattle, in the premature patient weighing less than 2.5 kg. Traditionally, these low birth weight infants underwent gastrostomy and fistula ligation followed by repair after a period of weight gain. However, since 1980, of the 31 neonates weighing 2.5 kg or less with type-C TEF, a primary repair was performed in 22 within the first five days of life. There were no mortalities and only one subclinical anastomotic leak. All anastomoses were done extrapleurally in a single-layer interrupted fashion. Staged repair was elected for nine neonates. Seven had severe respiratory distress requiring mechanical ventilation and two in the staged group expired prior to repair. The survival for the entire group of 31 neonates was 94%. Primary repair may be performed safely in the premature neonate. The presence of severe pulmonary disease is the critical factor that might necessitate a staged repair.