We report a rare case of a 24-year-old woman who presented during her second trimester of pregnancy with a presumptive diagnosis of right-sided xanthogranulomatous pyelonephritis (XGP). Despite attempted conservative management, the patient ultimately required a right nephrectomy without complications to either the patient or fetus. Pathology confirmed the diagnosis of XGP of the right kidney. Herein, we present a case report and a review of the literature concerning XGP and pregnancy.