A new case of pseudo-diastrophic dysplasia in a boy born to consanguineous parents is reported. The patient developed neurologic (hydrocephalus) and respiratory anomalies and died at the age of 11 months of an unknown cause. Features that distinguish this chondrodysplasia from diastrophic dysplasia include decreased height of vertebral bodies and anomalies of the pelvis with short iliac wings and a horizontal orientation of the internal spine of the acetabular roof.