Granulomatosis with polyangiitis (GPA) is a rare necrotizing autoimmune disease involving small vessel vasculitis. Pregnancies with GPA have increased rates of obstetric complications including pre-eclampsia. Differential diagnosis of GPA flares up and pre-eclampsia may be difficult and necessitates careful clinical practice. A 26-year-old pregnant woman with GPA was referred for hypertension. The absence of GPA signs and symptoms, negative anti-neutrophil cytoplasmic antibody titer and the presence of clinical and laboratory findings supported the diagnosis of pre-eclampsia rather than a GPA flare-up. The newborn was delivered via cesarean section at the 30th gestational week due to severe superimposed pre-eclampsia. Pathological examination of the placenta demonstrated the presence of chorangiosis and focal placental infarcts. GPA should be considered as a risk factor in pregnancy and requires careful clinical management to have good gestational outcome. Physicians should be vigilant regarding gestational diabetes and pre-eclampsia as well as GPA flare-up.