Myotonic dystrophy is a hereditary neurological disease. While cardiovascular complications have been recognized, recent reports indicate that it increases the risk of deep vein thrombosis and pulmonary embolism (DVT/PE) compared to other dystrophies and healthy individuals with dyspraxia and a history of DVT as risk factors. It is important to consider myotonic dystrophy in patients with repeated idiopathic DVT/PE and to provide appropriate medical consultation.