We describe a male patient presenting with Currarino triad: a recto-urethra
l fistula, sacral bony deformity, and a presacral teratoma. Clinical screen
ing of his family revealed three additional cases with incomplete forms of
this association. Cytogenetic findings in the patient and his mother were n
ormal. This case suggests that the occurrence of an anorectal malformation
together with a sacral bony deformity should raise a physician's index of s
uspicion for associated presacral tumors, and that screening of the patient
's family members with sacral radiographs is necessary.