INTRODUCTION Lung agenesis is an infrequent pulmonary malformation; it may be bilateral, which is incompatible with life, or unilateral presenting in a symptomatic or an asymptomatic manner. The estimated frequency is of 1 out of 15,000 living newborns. It occurs in the embryological stage of pulmonary development, due to a disruption in the differentiation and branching of the foregut. It typically presents other abnormalities, such as facial, auditory, cardiovascular, skeletal and genitourinary malformations. The usual clinical