Bronchopulmonary foregut malformations are a group of rare congenital anomalies affecting the respiratory and upper gastrointestinal tract. The rarity of these cases means their embryological origin continues to be a source of controversy. We present the case of a female infant, born at term with a malformed right arm, an absent right kidney and aplasia of the right lung. Although initially asymptomatic she presented at 5 months of age in severe respiratory distress. An upper gastro-intestinal contrast study demonstrated a right broncho-esophageal fistula. At surgical resection it was discovered that the right main bronchus ended abruptly just beyond the carina, with total aplasia of the right lung. A bronchoesophageal fistula originating from the lower third of the esophagus communicated with a sequestered right lobe. The lobe was removed and the fistula ligated. The infant remains well 13 months post surgery. This represents only the twelfth case of this rare form of bronchopulmonary foregut malformation. The associated renal and limb malformations make this case unique and may add weight to the theory that the underlying insult to the developing lung is vascular in origin.