Neuroborreliosis may be caused by different species of Borrelia burgdorferi (BB) and the clinical presentation of neuroborreliosis in children may differ between geographical areas due to occurrence of different BB genospecies. The aim of this study was to evaluate the clinical characteristics in children with neuroborreliosis in an endemic area of Scandinavia. During 1996-2006, children with suspected neuroborreliosis referred to Stavanger University Hospital were investigated by a standard procedure including a lumbar puncture. A total of 143 children were diagnosed with neuroborreliosis, and all cases were diagnosed from April to December. The most common clinical presentations were symptoms of mild meningitis (75%) and/or facial nerve palsy (69%). Radicular pain was present in only 10 children. In all but 5 children, laboratory signs of meningitis were present. Erythema migrans preceded the neurological symptoms in only 27% of the children. In conclusion, we have found that in an endemic area of northern Europe, meningitis is present in the majority of children with neuroborreliosis, and that symptoms of a mild meningitis or facial nerve palsy are the most common presentations.