This paper presents data from 23 British herds investigated between 1991 and 2007 where neurological disease in calves was caused by bovine viral diarrhoea virus (BVDV) infection. A variety of clinical signs, most commonly tremor or trembling, were apparent in the calves from birth, and most were recumbent or unable to stand unsupported. Severe diffuse neuraxial hypomyelination was present in all of the calves, and immunohistochemistry revealed cerebral neuronal labelling consistent with congenital persistent pestivirus infection in each brain. BVDV was detected in peripheral blood samples from eight of 15 calves tested using an antigen ELISA, and was isolated in culture from samples of viscera, brain or blood collected from 17 of 24 affected calves. TaqMan RT-PCR for pestivirus RNA was positive for BVDV-1 in all six calves tested. Six of the virus isolates on which molecular classification was carried out, obtained from calves in four of the herds, were identified as BVDV-1a, while three isolates from one affected and two unaffected calves on a fifth farm were confirmed as BVDV-1b.