Neu-Laxova syndrome is a rare form of congenital malformation characterized by intrauterine growth retardation, microcephaly with bizarre facial features, short neck, apparent edema, scaly skin, and perinatal death. Chromosomal analysis in reported cases has revealed a normal karyotype, and an autosomal recessive transmission has been postulated. We present a case of Neu-Laxova syndrome. The pathologic features and the prenatal radiographic appearance are described.