Kyphotic deformity arising from the failure in formation of a vertebral body is an uncommon condition showing late complications of gross spinal angulation, paraplegia, impaired bladder function and cardiopulmonary deficiencies. Congenital hemivertebra constitute approximately 6% of anomalies associated with congenital spinal deformities. The natural course of this disease remains unpredictable, especially regarding the development of neurological impairment. Only a few numbers of patients with severe kyphosis due to congenital dorsal hemivertebra have been reported. We present a 40-year-old man with severe thoracic kyphosis. Gait difficulty due to paraplegia occurred at the age of fifteen with progressive development of the right lower limb pain and later impairment of bladder function. He had no cardiopulmonary deficiencies. Radiological findings showed a wedge shaped dorsal hemivertebra and cord compression at the eleventh level of thoracic vertebra. In view of the poor prognosis of surgical intervention he was fitted with an Jewett brace so that further neurological impairment was avoided. We report a rare case of congenital kyphosis due to dorsal hemivertebra in adult with progressive neurological impairment.