Myotonic dystrophy is a multisystemic disorder inherited as an autosomal dominant trait. The secondary gastrointestinal disturbances are not well recognized. Two cases of myotonic dystrophy in a 51-year-old and 39-year-old woman with gastrointestinal symptoms disturbances are reported. One patient presented motor dysfunction in the colon and esophagus, organoaxial gastric volvulus and cholestasis. The second patient developed an acute colonic pseudo-obstruction. The patient improved with cisapride. The digestive manifestations of myotonic dystrophy and the treatment are reviewed.