Benign paroxysmal torticollis is a condition characterized by attacks of head-tilting often accompanied by lateral curvature of the trunc. The first onset of these attacks usually occurs during the first months of life and they recur with a remarkable periodicity for 6--12 months after which time they gradually subside in intensity and frequency. As a rule the attacks have ceased completely before the age of 2 years but in some cases they continue in a modified form as attacks of ataxia. The aetiology is unknown and there is no evidence of bilateral peripheral vestibular disturbances as has been suggested in earlier reports. The ataxia seen in some cases rather suggests a dysfunction of the cerebellum or of the vestibulo-cerebellar connections. Four own patients with this syndrome are presented and discussed.