Babies with major malformations were identified during the Jamaica Perinatal Morbidity and Mortality Survey. They were in 96 (8.6 por ciento ) of 1112 perinatal and neonatal deaths coming to necropsy and in 25 (2.35 por ciento ) of 1085 other deaths. The central nervous system was not most commonly affected, followed by the renal, gastro-intestinal and cardiovascular system in decreasing order of frequency. Many infants had abnormalities in more than one system and 10 malfomation syndromes/sequences were identified. Although at the present time, major malformations make only a small contribution to perinatal and neonatal mortality in Jamaica, its importance will increase as there is a fall in deaths related to perinatal asphyxia, currently the major cause of perinatal mortality in Jamaica. Although many malformations are currently untreatable, it is important to take account of gastro-intestinal defects when planning surgical services for the newborn, since these are the most easily remediable.