Introduction: Acute kidney injury (AKI) secondary to retroperitoneal fibrosis (RPF) is a rare disorder characterized by excessive retroperitoneal fibrosis leading to urinary tract obstruction. Data specifically addressing patients presenting with severe AKI and their long-term renal outcomes are limited. Moreover, predictors of renal recovery and progression to chronic kidney disease (CKD) or end-stage kidney disease (ESKD) remain poorly defined, partly due to heterogeneity in study populations, therapeutic strategies, and follow-up protocols. We conducted a retrospective study to comprehensively analyze the clinical, biological, radiological, and therapeutic characteristics of this condition and its renal outcomes. Methods: We retrospectively included 24 patients with AKI associated with RPF. Clinical, laboratory, imaging, histological, and therapeutic data were extracted from medical records for detailed analysis. Results: The mean age at diagnosis was 50.4 years, with a male predominance. The most frequent presenting symptoms were low back pain (83.3%) and general malaise (79.2%). All patients exhibited renal impairment, with 59% classified as stage 3 AKI. Imaging revealed ureteropyelocal dilatation in all cases, and poor renal differentiation in 23.8%. Histology showed non-specific fibro-inflammatory changes in 27% of cases. Therapeutically, 62.5% required emergency urinary drainage, and 29% required renal replacement therapy. Corticosteroids were administered to 83.3% of patients for a mean duration of 13.8 months. Relapse occurred in 33% during follow-up, and 70.8% progressed to chronic kidney disease. Conclusion: AKI due to RPF is a rare but severe condition, predominantly affecting middle-aged men. This study provides valuable insights into the clinical course, management, and long-term renal outcomes of patients with severe AKI due to RPF. Early recognition and timely intervention are critical to prevent progression to CKD, and our findings may help identify patients at higher risk of poor renal recovery