The myotonic dystrophies (DMs) are autosomal dominant disorders, subdivided by their genetic defect into DM1and DM2 and characterized by gradually progressive muscle weakness, myotonia, cataracts, endocrine disturbances and functional abnormalities of the cardiorespiratory and gastrointestinal tract. We report the unusual coexistence of spinal cord meningioma in a patient of myotonic dystrophy. As per our knowledge, this is the second case of a meningioma reported with myotonic dystrophy in the literature.