Dermatomyositis is a rare autoimmune disorder afflicting an estimated 6-10 million people worldwide that has a distinct clinical entity identified by a characteristic skin rash and inflammatory myopathy. It can be combined with other connective tissue diseases such as systemic sclerosis or systemic lupus erythematosus. In addition to primary disturbances of limb muscle and skin, extramuscular manifestations include dysphagia, cardiac conduction abnormalities, interstitial lung disease or subcutaneous calcifications. Dysphagia occurs in 12%-54% of those with dermatomyositis, manifests gradually over weeks to months rather than in acutely, and is diagnosed with high-resolution esophageal manometry and video-fluoroscopic monitoring during swallowing. We report a case of dermatomyositis presenting initially with acute oropharyngeal dysphagia and review the available swallowing studies and related literature.