This report describes a case of severe limited Wegener's granulomatosi
s (WG) presenting in the third trimester of pregnancy with pansinusiti
s and necrotizing pneumonitis. The patient was treated successfully wi
th a combination of corticosteroids and cyclophosphamide (CYC). The ou
tcomes in the mother and the newborn were excellent. In a review of th
e English-language literature, we found 10 similar cases of WG with 13
pregnancies, WG occurring during pregnancy may have a more aggressive
course and may require more aggressive treatment compared with WG occ
urring at other times. The treatment options for WG in pregnancy are d
iscussed.