AIMS To identify the clinical characteristics of fetal abdominal wall defects managed at a single institution and to provide information regarding the most likely clinical course of the affected fetuses. METHODS A retrospective review was conducted of 44 fetuses prenatally diagnosed with abdominal wall defects at Kyushu University Hospital between 1990 and 2006. RESULTS A total of 11 cases of gastroschisis and 33 cases of omphalocele were found. Preterm delivery was observed in eight of 11 fetuses (72.7%) with gastroschisis and in 14 of 27 fetuses (51.9%) with omphalocele who were not artificially aborted. Intrauterine growth restriction (IUGR) was identified in four of 11 fetuses (36.4%) with gastroschisis and in 13 of 27 fetuses (48.1%) with omphalocele who were not aborted artificially. There was a high rate of associated structural anomalies with omphalocele (26/33). Karyotypic abnormalities were observed in eight of 29 tested fetuses with omphalocele (27.6%). The prognosis for a chromosomally normal fetus with omphalocele appeared to largely depend on the associated structural abnormalities and the gestational age at delivery. Despite the frequent presence of IUGR, the outcome of neonates with gastroschisis was good. CONCLUSIONS The possibility of preterm delivery and IUGR should be considered during the management of both omphalocele and gastroschisis. For the management of omphaloceles, chromosomal abnormalities and respiratory insufficiency after birth must also be considered. Timely prenatal diagnosis and the subsequent identification of associated anomalies will improve patient care and will enable clinicians to provide appropriate counseling about the expected course.