A 22-year-old woman, Gravida 2 Para 1 Living 1 with 35 weeks of gestation with previous one lower segment cesarean section (LSCS) was referred to our department as a case of 35 weeks pregnancy with GPA with nephritic syndrome in remission. Her past history revealed the disease course, which started 1 year back with painless skin lesions (vesicles and papules) over upper and lower limbs, with recurrent bilateral flank pain and occasional episodes of syncopal attack. Further she developed hemoptysis and breathlessness requiring critical care admission at private hospital in Lucknow, India. On evaluation then, revealed presence of hypertension, perinuclear ANCA (P-ANCA) positive, hemoglobin (5.4 g/dL) suggesting severe anemia, her renal function test (RFT) was impaired, chest X-ray showed bilateral pneumonic consolidation, based on above findings, a diagnosis of ANCA-associated vasculitis (AAV) with pneumonia with severe anemia with acute kidney injury was made. The skin lesion was biopsied too, the histopathology however, had no features of vasculitis. The induction therapy commenced with intravenous pulse administration of CYC and methylprednisolone. In addition, she was also started on antihypertensive therapy with tablet amlodipine and losartan and received four units of packed cell transfusion. She responded well to the treatment and was discharged on tapering doses of oral prednisolone and maintenance therapy with oral azathioprine (AZA) and antihypertensives to continue. Besides this she also gave history of 6 months of antitubercular (ATT) therapy in view of pulmonary tuberculosis few months before conception. She conceived during remission phase and was continued on AZA and amlodipine. At around 30 - 32 weeks, patient herself stopped AZA and Amlodipine. Later at 35 weeks, she was referred to All India Institute of Medical Sciences (AIIMS) nephrology department in view of nephritic syndrome, and AZA was restarted, and she was further referred to our department. She was admitted in view of high-risk pregnancy. All her previous antenatal investigations in the current pregnancy were found to be normal including the anomaly scan. On admission, detailed clinical and laboratory evaluation was done. Her blood pressure was found to be normal. The laboratory evaluation revealed ANCA was negative, RFT values (blood urea nitrogen (BUN) 34 mg/dL, serum creatinine 2.57 mg/dL, urea 52 mg/dL, uric acid 8.3 mg/dL, urine albumin to creatinine ratio (ACR) 1,525.76, urine creatinine 21.19 mg/dL and urine microalbumin 323.52 mg/L. Fundoscopy revealed normal finding, ultrasound was suggestive of fetal growth restriction (FGR) and abnormal umbilical Doppler changes. The pregnancy was terminated at 37 weeks owing to worsening renal function (on serial RFT) with previous LSCS with FGR with delivery of an alive healthy female baby of birth weight 2.05 kg with Appearance, Pulse, Grimace, Activity, and Respiration (APGAR) score of 10 in 1 min and 5 min. She remained in remission in her post-partum period and was discharged on maintenance therapy with oral AZA in consultation with nephrologist. At Present, both the mother and the baby are doing well.