Society of Diabetic Nephropathy Prevention Journal of Nephropathology 2251-8363 6 4 2017 10 01 Rapidly progressive nephromegaly in a neonate with autosomal recessive poly cystic kidney disease 363 367 10.15171/jnp.2017.60 EN Mitra Naseri Nona Zabolinejad Syed Ali Alamdaran Reza Shojaeian Journal Article 10.15171/jnp.2017.60 2017 03 05 2017 07 03 Background: Autosomal recessive polycystic kidney disease (ARPKD) associates with significant renal and liver-related morbidity and mortality in children. Rarely severe enlargement of kidneys necessitates nephrectomy and initiating dialysis. Case Presentation: A 5-day-old newborn boy with prenatal ultrasonography suggestive of ARPKD admitted in the hospital due to anuria from first day of life. One week after admission, left nephrectomy was conducted because of severe enlargement of kidneys accompanied by respiratory distress and repeated vomiting. Patient was placed on peritoneal dialysis. Pathology of kidney revealed cystic lesions consisted of dilated collecting duct, with lack of involvement of glomeruli and other parts of the nephrons. Patient died at age 17 days with respiratory hemorrhage. Conclusions: Our case is interesting because of progressive increase in kidney sizes requiring nephrectomy. Such huge nephromegaly in ARPKD has not been reported yet.