Aim: We present a case wherein the information obtained from autopsy examination was of critical importance to a doctor and a family. Introduction: A 24-year-old,multipara,delivered a term born male baby with a birth weight 3100gr and body length 46cm.Soon after birth the neonate developed signs of POSTER SESIJA 63 MATERIA MEDICA • Vol. 34 • Issue 1, suplement 1 • april 2018. a respiratory insufficiency and died within 2 hours.Anamnestic data from the mother revealed uncontrolled pregnancy. Material and Methods: Standard autopsy technic and standard procedure of paraffin embedded section routinely stained with H E was performed. Results: At autopsy,the external examination revealed characteristic facial features suggestive of Potter s face including posterior rotated low-set ears,flat nose,widely separated eyes,micrognatia and short neck. Autopsy revealed presence of bilateral hypoplastic lungs with total weigh of 28g,less than the expected range (normal 49g).On dissection lungs were airless, non-crepitant and sank in the water. Histologically findings were consistent with a diagnosis of pulmonary hypoplasia.The abdominal cavity was completely filled with markedly symmetrical enlarged kidneys.Total weight of both kidneys was 156g (normal 25g).On the dissection section showed multiple small cysts measuring 1-3 mm in size,completely replacing the cortex and medulla giving it a spongy like appearance and typical honeycomb structure.On microscopical examination we found cysts uniformly lined by cuboidal to flattened epithelium. Zaključak: We consider that this is a Potter Syndrome Type I due to Autosomal Recessive Polycystic Kidney Disease and is linked to a mutation in the gene PKHD1.2. Through this case,we are aware of the importance and benefit of autopsy,although the trend of autopsies in the world is decreasing.