We present a rare case of congenital cystic adenomatoid malformation (CCAM) type III (mycrocystic) in young, 19 years old primigravida. Diagnosis has been accomplished by ultrasound examination in 26th week of gestation. Hyperechogenic, mycrocystic mass was found in left pulmonary lobe with contra lateral displacement of mediastinum, hypoplasia of the right lung and extensive polyhydramnios. Prenatal therapy was based upon a serial of amniodrainage procedures and pharmacological inhibition of amniotic fluid production and uterine activity. Spontaneous preterm labor occurred at 34 weeks of gestation, Diagnosis was confirmed postnatal. Infant was ventilated due to respiratory insufficiency and was operated at the age of 24 hours. Lethal outcome occurred during the second day due to heavy respiratory insufficiency provoked by pulmonary hypoplasia. We discuss about types of a disease, prenatal diagnostic and therapeutic possibilities and pregnancy outcome.