We report the case of a 32-year-old patient with Wegener's granulomatosis (WG) associated with a (biopsy - proven) renal inflammatory pseudotumour (IPT) of the left kidney treated by a partial nephrectomy, glucocorticoids and immunosuppressive drugs, in whom a relapse of renal IPT was found 6 years after the diagnosis of the first IPT. The originality of this observation lies in the fact that a relapse of IPT has never been described and also in the fact that complete regression of the IPT relapse was obtained with immunosuppressive treatment, while renal IPTs are currently treated by total or partial resection of the kidney. Finally, we discuss the potential benefits of an integrated 18fluorodeoxyglucose PET/CT for the follow-up of WG, since this imaging technique contributed to the management of the present case.