We present an unusual case of extralobar pulmonary sequestration associated with hyaline membrane disease (HMD) that caused the death of a premature baby in the first day of life. The sequestered parenchyma was nourished by an aberrant aortic vessel. Notable was the presence of typical HMD in all the lung parenchyma perfused by the pulmonary artery; the sequestered lung tissue presented a dysplastic structure compatible with CCAM. A few similar cases have been found in the literature. In all of the reported cases there are morphologic aspects characteristic of HMD in the portions normally receiving blood from the pulmonary artery. These findings suggest the importance of the blood pulmonary circulation in the pathogenesis of HMD, whose exact causes are not fully known.