RESUMO
The pulmonary hypoplasia with bronchoesophageal fistula is a rarely-seen disease which can be confused with total pulmonary agenesis. This case study concerns to one day old male newborn which was hospitalized in our hospital with respiratory difficulties, accompanied by pulmonary opacity of the right side. After having carried out diagnosis studies, including bronchography, we concluded that it was a case of pulmonary agenesis. The patient died from persistent pulmonary hypertension. The anatomopathologic diagnosis, was right pulmonary hypoplasia with bronchoesophageal fistula. This pathology originated during early embryonic stage when the bronchoplastic and esophagoplastic tissue are incompletely separated an thus a fistula between the esophagus and the principal bronchus is formed. The presence of a fistula could not be proven by bronchography. However, esophagography or esophagoscopy would have proven the presence of the fistula.