A 2-month-old girl was diagnosed with a febrile urinary tract infection. Subsequent studies revealed a right renal duplication anomaly with a poorly functioning upper pole moiety subtended by an intravesical ureterocele. The patient was also found to have hyperkalemia, hyponatremia, and elevated serum aldosterone. After antibiotic therapy, the urinary tract infection resolved, although the electrolyte and hormonal abnormalities persisted. At 4 months of age, a right upper pole heminephrectomy was performed. The patient's electrolyte and hormonal levels normalized. This case represents an unusual case of pseudohypoaldosteronism presenting with urinary tract infection and obstructed upper pole moiety that resolved after surgical intervention.