The success rates for pregnancies in women with systemic lupus erythematosus (SLE) have improved over the years: however, pregnancy for women with active, serious, organ-threatening lupus continues to be a challenge. Preeclampsia and HELLP (hemolysis, elevated liver enzymes and low platelets) syndrome may complicate pregnancy especially in the setting of advanced maternal age, underlying SLE and chronic renal disease. We report the pregnancy course and outcome for a 35-year old woman with active lupus nephritis at the time of conception who developed severe preeclampsia and HELLP syndrome. The infant was delivered at 26-5/7 weeks gestation, which, associated with intrauterine growth retardation, led to a birth weight of only 470 g. We have reviewed the relevant literature for similar cases of prematurity, very low birth weight, and preeclampsia in the setting of underlying lupus in Medline between 1986 and 2006. This report represents the lowest birth weight pregnancy survival in a lupus patient and the first case of a survival in the second trimester with preeclampsia and HELLP syndrome.