We retrospectively evaluated maternal and fetal outcomes in a series of women with von Hippel-Lindau disease. Data resulted from 56 pregnancies in 30 women. Symptoms related to von Hippel-Lindau disease occurred in 3 of 56 pregnancies. In this unselected group of patients with von Hippel-Lindau disease, most pregnancies had a favorable outcome with a 96.4% survival rate for fetuses and a 5.4 % von Hippel-Lindau-specific morbidity rate in the mothers.