Xanthogranulomatous pyelonephritis (XGP) is a rare, chronic inflammatory renal disease typically caused by bacterial infections. We hereby report a rare case of bilateral XGP secondary to Candida albicans in a 31-year-old female with multiple autoimmune disorders. The patient presented with nonspecific abdominal discomfort, painful urination, and generalized weakness. Computed tomography (CT) of the abdomen revealed bilateral hydronephrosis, renal enlargement, and chronic changes consistent with XGP. Despite aggressive antifungal therapy, her renal function progressively deteriorated, ultimately leading to multi-organ failure and death. This case underscores the importance of maintaining a high index of suspicion for XGP in immunocompromised patients to facilitate early diagnosis and potentially improve outcomes. Additionally, it highlights the unique diagnostic challenge posed by C. albicans as a causative pathogen in XGP, emphasizing the need to consider fungal infections in the differential diagnosis to avoid delays in treatment.