Aims Report the case of death of a premature infant with congenital malformations exposed to cocaine during pregnancy. Case history Emergency services are called for a delivery at home. The child, in cardiopulmonary arrest, died during the transfer to hospital. Gestational age was estimated at 33 weeks of amenorrhoea. The examination shows multiple malformations. Results Peripheral blood tests revealed morphine at a concentration of 210 μg/L, as well as cocaine, benzoylecgonine, and ecgonine methylester at respective concentrations of 2.5, 645, and 35 μg/L. Morphine concentration is compatible with those described in preterm infants and corresponds to the administration of morphine by emergency services. Cocaine and metabolites concentrations are consistent with those measured in the cord blood of exposed newborns. Pathological examination reveals hyaline membrane disease. No genetic diagnosis is requested. Discussion/Conclusion If the death seems to be directly caused by pulmonary immaturity, the responsibility of cocaine in the induction of labor and malformations has to be discussed. While the risk of prematurity in cocaine-exposed fetuses is accepted, its teratogenicity is controversial. In this case, some of the malformations are consistent with those described in the literature, and there is no evidence of a genetic pathology. However, a genetic diagnosis and analysis of the mother's hair would have made it possible to rule out a genetic cause and to document exposure to cocaine during the first trimester of pregnancy. In conclusion, it seems important in these situations to carry out all the necessary investigations to determine the exact cause of death.