Heterotopic glial tissue was seen within the lungs of a newborn with a cranial malformation and anterior spina bifida. Histological examination of the lung showed solid islands of glial tissue intermingled with pulmonary structures. Well developed bronchioles that were in continuity with the main bronchial tree could be identified within the nodules. The central cystic cavity of the nodules is a bronchial lumen probably dilated by the respiratory movements. Several hypotheses have been elaborated to explain the heterotopic glial tissue in lung. The presence of an anterior spina bifida suggests a migratory mechanism to explain the embryogenesis of this case.