Sacral agenesis is an uncommon condition characterized by absence of different segments of lumbar spine along with total or partial absence of sacrum. It does not have an established etiology but may be associated with insulin dependent diabetes mellitus in the mother. Motor deficits are present and correspond to the level of vertebral agenesis. Sensation is better preserved than motor function. Orthopedic deformities such as hip dislocation, flexion contractures, genu recurvatum, posterior compartment atrophy, scoliosis and so on are observed. Urinary and bladder dysfunction are constant and it can lead to fatal kidney damage, finally. We report one case of type IV sacral agenesis with review of literature.