Myotonic dystrophy is an autosomal dominant inherited disease of the skeletal and cardiac musculature that involves the pharyngeal and gastrointestinal smooth and striated muscles, resulting in velopharyngeal insufficiency, Swallowing difficulties, gastrointestinal motility disorders and anal incontinence. Gastrointestinal symptoms are found in a large proportion of patients suffering from this disease and may herald the onset of muscular disorders, in rare cases they are even the predominant feature of the disorder. We report on a 31-years-old patient with formerly undiagnosed myotonic dystrophy in combination with a non-rotation of the intestinal tract, an association of disorders that to our knowledge never has been reported before. Our patient was admitted as an emergency with signs of an acute abdomen with ileus, associated with acute aspiration pneumonia. Surgical intervention was avoided once the diagnosis of myotonic dystrophy had been confirmed and the patient was treated successfully by conservative therapy. A review of the literature indicates that conservative treatment of motility disorders of the bowel in patients with myotonic dystrophy is to be recommended.