A 30-year-old female achondroplastic dwarf developed a progressive gait disturbance erroneously attributed to her hydrocephalus and deformities of both legs. Her condition deteriorated into flaccid paraplegia with anal and urinary incontinence. CT revealed extreme spinal stenosis typical in achondroplasia (shallow vertebral body, short pedicles, and hypertrophy of intervertebral joints) together with disc protrusions. Wide laminectomy of the lumbar vertebrae resulted in complete amelioration of all the neurological deficits. Progressive paraplegia is a rare complication of achondroplasia; its early recognition and surgical treatment is very rewarding.