In a multicentre retrospective analysis of 94 ever-pregnant women with mixed connective tissue disease (MCTD) who tested positive for anti-U1RNP antibodies, 147 of the 203 pregnancies (72.4%) resulted in live births, 38 (18.7%) ended in a first trimester pregnancy loss and 18 (8.9%) ended in a stillbirth. The fetal outcomes were worse for women who also tested positive for antiphospholipid antibodies or had pulmonary or muscular involvement. Specific counselling was advised for patients with MCTD who are planning a pregnancy.