Myotonic dystrophy type 1 (DM1) is the most common form of adult muscular dystrophy. It is an autosomal dominant inherited disease with multisystemic involvement. Respiratory function is often affected and respiratory failure is the most common cause of death. Pulmonary embolism is a rare cause of respiratory failure in DM1 patients, so that the best anticoagulation strategy in these patients is still unclear. We describe the case of pulmonary embolism in a DM1 patient, in which pulmonary thrombus was completely resolved with oral dabigatran etexilate therapy.