Cervical spondylolisthesis is a rate vertebral anomaly that has not been associated with other major congenital abnormalities. In a 16-year-old girl, lower cervical spondylolisthesis was associated with a multiple nevoid basal cell carcinoma syndrome. The salient features of cervical spondylolisthesis include occipital headache, nuchal rigidity, torticollis, painful range of limited motion, hypesthesia, and depressed deep tendon reflexes. The treatment in the patient was anterior vertebral body fusion. Awareness of this associated problem and prompt treatment are essential for prevention of neurologic loss.