Based on a study of 16 fetuses that were diagnosed as having inencephaly, we discuss the correlation between sonographic and the pathologic findings in relation to the literature. Anencephaly was the most common anomaly associated with iniencephaly in the first trimester (100%). Other associated anomalies were encephalocele, meningomyelocele, micrognathia, omphalocele, gastroschisis, kyphoscoliosis, and upper and lower extremity abnormalities. History of neural tube defect and accompanying anomalies of iniencephaly such as anencephaly should be considered as potential diagnostic indicators of iniencephaly.