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Summary  Neonatal hyperkalemia and hyponatremia are medical conditions that require an emergent diagnosis and treatment to avoid morbidity and mortality. Here, we describe the case of a 10-day-old female baby presenting with life-threatening hyperkalemia, hyponatremia, and metabolic acidosis diagnosed as autosomal dominant pseudohypoaldosteronism type 1 (PHA1). This report aims to recognize that PHA1 may present with a life-threatening arrhythmia due to severe hyperkalemia and describes the management of such cases in neonates.   Learning pointsPHA1 may present with a life-threatening arrhythmia.    Presentation of PHA can be confused with congenital adrenal hyperplasia.    Timing and appropriate medical management in the critical care unit prevented fatality from severe neonatal PHA.

Pseudohypoaldosteronism in a neonate presenting as life-threatening arrhythmia