We report a case of autosomal recessive polycystic kidney disease (ARPKD). A presumptive diagnosis was made after a late-term prenatal ultrasound revealed hypoplastic lungs, massive polycystic kidneys, and oligohydramnios. A full-term baby girl was delivered vaginally. Respiratory distress required intubation. Twelve hours after birth, she underwent bilateral nephrectomy and peritoneal dialysis catheter placement. The average kidney size was 150 g and 9.25 cm. Pathologic examination confirmed ARPKD. Peritoneal dialysis was started on the third day of life. The baby had no gross neurologic deficit. At 6 months of age, she was growing well, and the mother was a candidate to be a living-related kidney donor.
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