Rectal atresia (RA) with a normal anus is a rare anomaly mostly descri
bed as part of a series of anorectal malformations. Most authors belie
ve it to be an acquired lesion with a vascular genesis. One of the arg
uments quoted is the lack of other congenital anomalies. Several opera
tive procedures are recommended for this lesion. We describe four pati
ents with RA who had other significant congenital anomalies; two other
cases were found in the literature. A lack of other congenital anomal
ies in patients with RA does not seem to be a strong argument for an a
cquired lesion, All four patients were treated by a posterior sagittal
approach with good functional results.