[Pulmonary valve agenesis and giant lobar emphysema. A not uncommon association].

A case of neonatal giant lobar emphysema secondary to a complex cardiac anomaly with pulmonary valvular agenesis is presented. Clinic and radiologic findings are sufficient to suggest diagnosis. Right heart catheterization is required to demonstrate the pulmonary valvular agenesis. Such an investigation should be recorded when observed a giant neonatal emphysema. Is failure occurs in the neonatal period the prognosis of this association is poor.