Background: Systemic lupus erythematosus (SLE) is an autoimmune disease which involves multiple organs, including peripheral nervous system. Case presentation: We describe a 12-year-old boy with progressively worsening neurological symptoms as first manifestation. Legs pain, loss of balance, and lower extremity weakness were the reason for his admission in neurologic ward. The patient was started on intravenous immunoglobulin therapy due to the possibility of Guillain-Barre syndrome and acute inflammatory demyelinating polyneuropathy (AIDP). However, there was no appropriate response and he developed recurrent attacks of polyneuropathy again with diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Then, he received intravenous pulse of methylprednisolone for 5 consecutive days followed by oral prednisolone for 3 months. One month after withdrawal of corticosteroid he admitted again with the same manifestations. Rheumatologic workup revealed the presence of leukopenia, hemolytic anemia, hematuria, proteinuria, positive antinuclear antibodies, and ds-DNA antibodies. On the basis of the American College of Rheumatology and Systemic Lupus International Collaborating Clinics Classification Criteria for SLE, the patient had underlying diagnosis of SLE. Eventually, he was treated by the pulse of methylprednisolone and cyclophosphamide, and oral hydroxychloroquine and prednisolone. His neurological and physical symptoms improved and complete neurological recovery occurred several months later. Conclusion: SLE and AIDP/CIDP are different entities, but ADP/CIDP can be part of the neurologic manifestations of the SLE. Although the association between AIDP/CIDP and SLE is very rare especially as a first manifestation of SLE, it should be early recognized for rapid appropriate treatment.