Background: India faces the biggest newborn health challenge of any country in the world as one fourth of global neonatal deaths occur in India. The common cause of neonatal mortality in India are asphyxia, prematurity and low birth weight, infections like pneumonia and gastroenteritis and a variety of surgical problems. It has been observed that improved level of newborn care can bring down the mortality rates. A study of Neonatal Intensive Care Unit (NICU) was undertaken at SKIMS Srinagar to determine the case fatality among the neonates at a Tertiary Care Teaching Hospital in Jammu & Kashmir. Material and Methods: A descriptive case series hospital based prospective study was conducted over a period of one year from with effect from 1st January 2013 to 31st December 2013 in NICU of SKIMS Srinagar by administering a predesigned standardized questionnaire proforma to one or both parents of admitted neonates in a NICU of Sher-i-Kashmir Institute of Medical Sciences (SKIMS) Srinagar, Jammu and Kashmir-India. Results: Neonatal Jaundice (NNJ) (26.7%) was the most common cause of morbidity and admission to NICU followed by Septicemia (19.1%) Prematurity (12.5%), Birth Asphyxia (7%) and Respiratory Distress Syndrome (RDS) (5.7%). It was observed that out of 1017 neonates admitted most were discharged (90.07%) whereas as 9.73% expired and only 0.2% left against medical advice (LAMA). Being insignificant in numbers the two neonates that left against medical advice (LAMA) were excluded from further analysis for the case fatality rate. It was observed that the case fatality rate was maximum in MAS (33.3%) followed by Congenital Anomalies (23.1%), Prematurity (18.9%), Birth Asphyxia (15.5%), RDS (15.5%), & Septicemia (9.3%). TheChi-squared test (χ2) static value was 89.49585 and the p-value was less than 0.0001 indicating that type of disease is very strongly associated (p<0.0001) with the case fatality of a neonate. Conclusions: The case fatality rate was maximum in MAS (33.3%) followed by Congenital Anomalies (23.1%), Prematurity (18.9%), Birth Asphyxia (15.5%), RDS (15.5%), & Septicemia (9.3%) and thetype ofdisease is very strongly associated (p<0.0001) with the case fatality of a neonate.