Since 1973, seven infants treated at the University of Florida, Gainesville, for congenital anorectal anomalies have been found to have concomitant sacrococcygeal teratomas. As anorectal anomalies occur in approximately one in every 5,000 live births and sacrococcygeal teratomas are noted in one in every 40,000 live births, this presentation would suggest a prevalence that precludes a random association. The presence of significant anorectal strictures in these patients resulted in a delay in the diagnosis of the presacral teratomas. As the frequency of malignant change in sacrococcygeal teratomas has been documented to increase proportionately with age, early diagnosis and excision of these lesions is essential. Our experience with these children suggests that the presence of congenital anorectal malformations in infancy should raise the physician's index of suspicion for associated sacrococcygeal teratomas.