A review of 14762 singleton live-born babies during a period of 6 years at the King Fahad Hospital, Al-Khobar, Saudi Arabia is made with a view to study the clinical pattern of easily identifiable congenital malformations among neonates. The incidence of congenital malformations in the study was 17.0/1000 live births. Major abnormalities were present in 74.4% and minor ones in 25.6% of cases. The anatomical organs most commonly affected were the central nervous system (CNS), musculoskeletal and renal defects followed by gastrointestinal and chromosomal defects. CNS anomalies accounted for 48.8% of the total defects, hydrocephaly, anencephaly and meningocele being the predominant lesions. The incidence of malformed babies in diabetic mothers was 7.8%. Multiple anomalies were present in 16.7% infants. Of the total 251 malformed infants, 38 died within the first week of life with a mortality rate of 15.1%, compared with the overall perinatal mortality rate of 12.2/1000 live births in the hospital during the period of study.