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Although neurological involvement occurs in about 10% of patients with mixed connective tissue disease (MCTD), acute transverse myelitis (TM) has only been described in seven cases of MCTD. We hereby report a case of 70-year-old white female with transverse myelitis complicating her underlying MCTD. Our patient presented with lower extremity weakness, loss of sensation and incontinence one year after her diagnosis of MCTD. Her work-up revealed an abnormal MRI, with findings consistent with TM. She had an excellent response to initial therapy with six cycles of monthly intravenous immunoglobulins and steroids, with subsequent maintenance on azathioprine. She had a good neurological recovery with mild residual sequelae only. On basis of this case report and review of literature, we recommend ongoing surveillance and reporting of this rare neurological presentation in MCTD.