Gastroschisis has an important impact on newborn (NB) mortality. In sub-Saharan Africa, the mortality rate remains high at around 60 per cent. This study analyzed the factors associated with lethality in newborns with gastroschisis in a reference hospital unit in Angola. An observational, descriptive, and cross-sectional, hospital-based study was carried out and included information from 361 clinical files of newborns with gastroschisis evaluated at the David Bernardino Paediatric Hospital from 1st January 2010 to 15th July 2021. The mean maternal age was 22.34±4.5 years (range:14-40 years); the mean birth weight was 2453.9±515.6 with a significant difference between discharged and deaths groups (p<0.001). The high lethality rate (96,1%) was associated with low birth weight (Exp β = 7.956; 95% CI 1.758 – 36.163; p =0.007), parity (Exp β = 0.296; 95% CI 0.096 – 0.919; p =0.035), as well as sepsis (Exp β = 4.827; 95%CI 1.119 – 20.814; p =0.035). The results show that the high lethality of gastroschisis is particularly associated with factors related to the NB such as birth weight, sepsis and parity.