Objective: Retroperitoneal fibrosis is a rare collagen vascular disorder of unclear cause. Both benign and malignant associations have been described, rendering differentiation of these entities of paramount importance because sinister pathology alters the diagnosis. Thus, a high level of diligence is required in the investigation of this condition, particularly in patients with concomitant systemic conditions.
Conclusion: Familiarity with the realm of imaging manifestations of retroperitoneal fibrosis is vital to ensure correct diagnosis and optimal treatment.