Caudal agenesis or caudal regression syndrome refers to a spectrum of malfo
rmations involving the lower spine, which can result in significant progres
sive or permanent neurologic defects. Caudal agenesis is frequently associa
ted with other congenital anomalies of the axillary skeleton, hindgut, and
genitourinary systems. Described in this paper is a patient who presented w
ith multiple clinical and radiographic findings consistent with caudal agen
esis. This case is unique in that there is to tal absence of the lumbar ver
tebral column with an intact spinal cord and bony sacrum.