We report a case of Wegener's granulomatosis (WG) who very well responded to the combination strategy of therapeutic plasma exchange (TPE) and immunosuppression. The patient was a 38-year-old female, diagnosed with severe form of WG. A total of seven cycles was performed with 1.3 total plasma volumes (TPVs) on every alternate day. Standard induction therapy was also started that comprised of a combination of 500 mg intravenous (i.v.) cyclophosphamide and methylprednisolone 1 g slow i.v. daily for 3 days followed by oral prednisolone 60 mg daily for 4 weeks. After seven cycles of TPE, the patient improved and hence TPE was stopped. © 2020 Asian Journal of Transfusion Science.