A rare clinical case of a newborn boy with a diagnosed Potter sequence is presented. The diagnosis was made based on polycystic dysplasia of the kidneys, cysts in the liver, hypoplasia of the lungs and characteristic external signs due to critical oligohydramnios. The child’s parents were closely related, which suggested an autosomal recessive form of the disease. The newborn lived for 15 hours, after which the death, developed as a result of respiratory failure, was ascertained.