Background: Tracheoesophageal fistula is one of the most serious congenital anomalies among neonates and associated with high morbidity and mortality rates so it is require competent and skillful pre & post –operative care. Aim: This study aimed to assess nurses' performance regarding care of neonates having tracheoesophageal fistula. Research design: A descriptive research design was employed to fulfill the aim of the current study and answered the research question. Setting: This study was conducted at Neonatal intensive care units in children hospital affiliated to Ain Shams University and Sohag University Hospital. Sample: A convenience sample of 70 nurses who were working in the previously mentioned setting. Tools of data collection: A) predesigned questionnaire format. B) Observation check list. Results: Only fifth of the studied nurses had a good level of knowledge and more than two fifths of them had a competent practices regarding care of neonates having tracheoesophageal fistula. Conclusion: More than two fifths of the studied nurses had a poor level of knowledge and more than half of them had incompetent level of practices regarding care of neonates having tracheoesophageal fistula and also, there were a statistical significant relation and positive correlation between total nurses' characteristics and their practices. Recommendations: Periodic assessment of knowledge and practices of all nurses dealing or providing care of neonates having tracheoesophageal fistula. Emphasize the importance of continuous training on appropriate nursing intervention which should be designed according to actual needs of neonates having tracheoesophageal fistula.