A pancerebellar syndrome of subacute progression associated with cerebellar atrophy is highly suggestive of a paraneoplastic cerebellar degeneration (PCD). We describe a 27-year-old woman with systemic lupus erythematosus (SLE) that presented with a subacute pancerebellar syndrome. Serum and CSF anti-Yo, anti-Hu and anti-Ri antibodies were not found neither in blood, nor in CSF. Brain MRI showed a cerebellar atrophy. The cerebellar ataxia improved markedly following corticosteroids administration. This case probably demonstrates that an antineuronal antibody negative subacute cerebellar disease may not only be a manifestation of paraneoplastic disease, but that it can also be associated with SLE.