The treatment of autosomal recessive polycystic kidney disease (ARPKD) is evolving. Overall prognosis is difficult to determine, but most neonates without severe pulmonary hypoplasia will survive. It is difficult to predict which neonates with ARPKD requiring immediate ventilatory support have pulmonary hypoplasia incompatible with survival. Massively enlarged kidneys restricting diaphragmatic excursion can cause respiratory distress in addition to pulmonary hypoplasia. Three infants with ARPKD underwent bilateral nephrectomies and institution of peritoneal dialysis in an attempt to improve ventilatory status and assess long-term pulmonary function. Two children are alive at twelve and thirty months postoperatively and awaiting renal transplantation. One infant died of acute cardiorespiratory complications.