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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.