Background: Congenital malformations are a major health problem and highly contributed tornneonatal and child mortality in all Sub-Saharan Africa. However, there are limited andrninconsistent pieces of evidence on the survival status of congenital anomalies and theirrndeterminants in Ethiopia. rnObjective: to assess the probability of survivorship and its predictors in the first year of lifernamong babies with congenital anomalies. rnMethods: A retrospective cohort study design was used among infants with congenitalrnanomalies in Black Lion Specialized Hospital and Zewditu Memorial Hospital in Addis Ababa,rnregistered from 2014 to 2017 which are referred from Addis Ababa region health facilities. Datarnwere collected by reviewing the registration book and interviewing parents by visiting theirrnhomes using an electronic data collection tool. Data were analyzed using STATA version 14.rnThe actuarial life table and Kaplan Meier survival curve were analyzed to estimate time to deathrnwith a log-rank test to compare survival time between groups. To identify independent predictorsrnCox-proportional model was carried out and crude and adjusted hazard ratios with 95%rnConfidence Interval (CI) were used to determine statistical significance. rnResults: The overall survival probability of infants with congenital anomalies to 7 days, 28 days,rnand 1-year was 70.3%, 50.0%, and 46.5%. Infant 1-year survival probability with nervousrnsystem congenital anomalies had (40.9%), digestive system (42.5%), circulatory systemrnanomalies (43.3%), chromosomal system anomalies (60.0%), musculoskeletal system anomaliesrn(50.0%), respiratory system anomalies (75.0%), and urogenital system (93.3%). The medianrnsurvival time for those cases that died was 6 days. Survival of male infants and low birth weightrninfants was significantly poorer survival and those infants who had surgical intervention werernsignificantly better survival. rnConclusion: Survival of infants with congenital anomalies to one year is low. Effectiverninterventions are required to focus on nutritional counseling, birth weight, and early surgicalrnintervention to improve the survival probability of infants with congenital anomalies.