Abstract
Emphysematous pyelonephritis (EPN) is a critical and life-threatening necrotizing urinary tract infection, marked by gas formation within the renal parenchyma, collecting system or peri-nephric tissue and is extremely rare in children. Proteinaceous calculi are a rare type of kidney stones, reported in only 0.5% of nephrolithiasis cases, and rarely reported in the pediatric population. We present a case of a 16-year-old female with concurrent EPN and proteinaceous calculi with recurrent Escherichia coli urinary tract infection.