We describe a case of extra-lobar pulmonary sequestration with broncho-esophageal fistula in a newborn male who presented respiratory distress, cyanosis and feeding difficulties. The diagnosis was made with a swallowed contrast examination, nuclear magnetic resonance, digital subtraction angiography and confirmed by gross and histologic examination. The work of a multidisciplinary team was essential for an early diagnosis and the correct and effective treatment of this Cuncommon condition.