Background: 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.