We describe a patient with systemic lupus erythematosus (SLE) who presented with acute transverse myelitis. Magnetic resonance imaging (MRI) of the cervical spine demonstrated increased signal intensity and diffuse edema in the cervical cord. The patient had low titer of IgG antiphospholipid antibodies and hypocomplementemia. Cerebrospinal fluid analysis showed pleocytosis, increased protein and decreased glucose. Clinical improvement of the neurologic impairment was noted after high dose corticosteroids and intravenous bolus cyclophosphamide. A repeat MRI 12 days after the treatment was normal. MRI of the spine can be useful in the diagnosis and followup of patients with SLE presenting with an acute myelopathic syndrome. Early aggressive treatment may contribute to complete recovery of these patients.