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Severe hyperkalemia is a medical emergency and occurs due to a variety of underlying illnesses. We present a 7 day old neonate who presented with life threatening hyperkalemia due to pseudohypoaldosteronism type 1 (PHA1). The clinical picture resembled congenital adrenal hyperplasia (CAH). Very aggressive management including peritoneal dialysis was required to control hyperkalemia. It is important to differentiate PHA1 from CAH since the former does not respond to corticosteroid therapy and may require peritoneal dialysis for control of hyperkalemia. A discussion on the types, clinical course, and management of pseudohypoaldosteronism type 1 is presented.

Pseudohypoaldosteronism type 1: A rare cause of severe dyselectrolytemia and cardiovascular collapse in neonates