A first case of congenital rubella with evidence of oligoclonal IgG in serum and cerebrospinal fluid during early manifestation is reported. The mother contracted the rubella infection during the first month of gestation. In addition to the more frequent symptoms of rubella embryopathy there were signs of active meningoencephalitis in the newborn. As a rare eye defect, bilateral congenital glaucoma was observed. Cranial computed tomography gave evidence predominantly for white matter involvement. Elevated IgG levels and oligoclonal IgG were found in both cerebrospinal fluid and serum. By means of quantitative estimation, a local synthesis of IgG in the central nervous system could be demonstrated.