Abstract
Immunoglobulin G4 (IgG4)-related disease was described in 2003 and is known to affect almost all organ systems. Rarely, this disease can manifest in the retroperitoneum, which can lead to sequalae such as obstructive uropathy. Here we describe a case of IgG4-related retroperitoneal fibrosis leading to obstructive uropathy. The patient was managed with bilateral Double J (DJ) stenting and oral steroids. Following remission of the disease, the DJ stents were removed and the patient remained asymptomatic over the follow-up period. A high index of suspicion is required for diagnosis of this rare disease and timely management can lead to a positive outcome.