Objective      To investigate the incidence and clinical pathologic features of congenital malformation in fetuses and perinatal infants through anatomical pathological examination.       Methods      A total of 438 autoptical specimens of fetuses and perinatal infants were collected from the Departments of Obstetrics and Pediatrics in our hospital during January 2005 to December 2010. Their autopsy and clinical data were analyzed for congenital malformation and main causes of death of neonatal.       Results      The coincidence rate of clinical diagnosis and pathological anatomy diagnosis was 92%. The incidence of congenital malformation in autopsy for 438 cases of fetal and perinatal infants was 85.84% (376/438). The incidence of congenital malformations  was 17.35% （76/438） in neural system

and 16.44%（72/438） in circulatory system

followed by cleft of lip and palate（42/376）

motorial system（32/376）

urinary system（30/376）

respiratory system（25/376）

chromosomal disorder（26/376）and digestive system（20/376）. Among these 376 cases

the cases of single malformation accounted for 243 (64.63%)

while the cases of multiple deformities were 133 (35.37%).       Conclusion      Congenital malformation is the most important cause for mortality in fetuses and perinatal infants. Clinical pathological anatomical examination is a useful tool to diagnose congenital fetal malformation and to verify causes of fetal death.