Acute transverse myelitis (ATM) is a very rare manifestation of the central nervous system in systemic lupus erythematosus (SLE), especially in case of involvement of continuous segments (longitudinal myelitis). We describe a 12-year-old female with lupus correlated with transverse myelitis with a longitudinal involvement of the spinal cord (D2 to D10) at the onset of the disease. Despite the administration of an early aggressive therapy, the outcome proved to be unfavourable. After 2 years of follow-up, the child still complains of paraplegia, sphincter incontinency and ipo-paresthesias of both legs.