The literature data about systemic primary vasculitis and pregnancy are presented. The pregnancy in patients with systemic primary vasculitis is characterized by low maternal and fetal mortality (<5—10%) and high premature delivery rate (up to 40%). Pregnancy in patients with vasculitis should be considered as high risk pregnancy requiring careful planning and regular monitoring by obstetricians, internists or rheumatologists who have experience in such special cases. It is important to take into account maternal age and the time required to achieve the total remission. Pregnancy should be approved only in sustained vasculitis remission or in vasculitis with minimal activity for at least 6 months without or with few organ damages.