To present a case of successful pregnancy outcome in a granulomatosis with polyangiitis (GPA) patient with renal insufficiency. GPA, formerly known as Wegener's granulomatosis, is a rare necrotizing systemic vasculitis, presenting with classical clinical triad of manifestations involving upper and lower airway and glomerulonephritis. An association of Antineutrophil cytoplasmic antibodies with GPA has been established and the antibodies are present in most patients with active disease. Pregnancy with GPA is burdened with the risk of possible maternal and fetal complications, further leading to higher morbidity and mortality rate. Due to sparsity of studies of GPA in pregnancy, management needs to be individualised. Diagnostic workup should include serological markers, radiological and histopathological examination. Cyclophosphamide combined with prednisolone is the standard induction regimen. A 22-year-old woman, multigravida at 35 weeks of gestation was referred to our department owing to 1-year diagnosis of GPA. During active phase, the disease manifested as pneumonia and acute kidney injury and perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCAs) were positive. She received pulse therapy of injection cyclophosphamide and methylprednisolone as induction regimen, followed by tapering doses of oral prednisolone and azathioprine for maintenance therapy. The disease was in remission at the onset of pregnancy but had flare up at 34 - 35 weeks of gestation and she presented with renal dysfunction. Neither the disease nor the treatment adversely affected the pregnancy and she delivered a healthy baby at 37 weeks. The unpredictable disease course and complications at unexpected gestation appears to be a major variable to take into account when assessing the risk of pregnancy with GPA. Early diagnosis, monitoring and timely intervention resulted in favourable pregnancy outcome in our patient.