A case-control study was carried out to estimate risk factors for perinatal mortality in a referral hospital for high-risk pregnancies in Curitiba-PR. Sociodemographic, maternal, pregnancy and concept characteristics data were obtained from the hospital records of 316 cases and 316 controls from 2013 to 2017. A hierarchical multiple logistic regression analysis was performed, remaining in the final model variables with p < 0.05. The results show an increased risk of perinatal death in mothers with blood type B (OR = 2.82; 95%CI: 1.07-7.43), who did not undergo prenatal care (OR = 30.78; 95%CI: 4.23-224.29), fetuses with congenital malformations (OR = 63.90; 95%CI: 27.32-149.48), born under 28 (OR = 24.21; 95%CI: 1, 10-531.81) and between 28-31 weeks of gestation (OR = 6.03; 95%CI: 1.34-27.17) and birth weight below 1,000g (OR = 51.94; 95%CI: 4.31-626.46), between 1,000-1,499g (OR = 11.17; 95%CI: 2.29-54.41) and between 1,500-2,499g (OR = 2.75; 25-6.06). Concepts of pregnancies with premature outcome, low birth weight and the presence of congenital malformations are the main risk factors for perinatal death. On the other hand, adequate prenatal care is an important protective factor.