Myelopathy is a rare but serious central nervous system complication associated with systemic lupus erythematosus (SLE). Acute transverse myelitis is the most usual involvement of SLE-related myelopathy. We reported a 17-year-old girl who developed a very extensive SLE related transverse myelitis with longitudinal involvement of the spinal cordfrom C4 to the conus medullaris. There were motor and sensory loss of both lower extremities, and bladder dysfunction over the course of 5 days. She presented T9 paraplegia ASIA A. Her neurological dysfunction was not responded to treatment with methyprednisolone and cyclophosphomide.