Introduction: Congenital anomalies (CA) of the Central Nervous System (CNS) are birth defects of the physical structure of the brain or spinal cord that occur during fetal intrauterine growth, some are caused by genetic factors and other by environmental factors. This study aimed to: Assess the nurses' competency level regarding the care of infants with congenital anomalies of central nervous system. Design: A descriptive study was used to conduct this study. Setting: This study was conducted at Neurosurgery Department at Mansoura University Hospital. Subjects: A purposive sample composed of all available nurses (55 nurses) which are working in the previously mentioned setting. Tools for data collection: First tool: Pre-designed questionnaire sheet to assess nurses' knowledge about CA of CNS. Second tool: Competency Checklist to assess nurses' practice regarding the care of infants with CA of CNS. Third tool: Modified Likert scale to assess nurses' attitude toward infants with CA of CNS. Results: More than three quarters of the studied sample were females, and about two third of them had less than 5 years in experience. In addition, slightly more than half of them had good total knowledge, slightly more than half of them had incompetent total practice and slightly less than two thirds of them had positive attitude toward infants with CA of CNS. Also, there was statistically significant difference between the studied nurses' total level of practice and their age, educational level and years of experience. Conclusion: There was positive correlation between practices of the studied nurses and their total level of knowledge about CA of CNS. Also, it was concluded that nurses' knowledge, practices and attitude affected on nurses' competency level, in addition to that, sex of the studied nurses, their age, educational level and years of experience were considered as factors affecting their competency level in caring of infants with CA of CNS. Recommendations: Developing a simplified and comprehensive protocol including policy and procedure about care of infants with CA of CNS to be distributed for nurses in neurosurgery departments.