Statins are widely used and are currently the state-of-the-art treatment for primary and secondary prevention of cardiovascular disease. Although statins are generally well tolerated and present an excellent safety profile, adverse effects from muscle toxicity may occur in some patients. Statin-induced dermatomyositis (DM) is a rare adverse event associated with its use and very few fatal cases have been reported. We present the case of a 69-year-old man with early onset DM precipitated by a small dose of simvastatin. Despite immediate cessation of the agent and the use of systemic corticosteroids, the case took a very aggressive and fatal course. Such progression is extremely unusual for statin-induced DM. Despite the safety of statins, we highlight the importance of identifying potential side effects associated with this class of medications. We also emphasize the importance of correct diagnosis and close follow-up of patients with statin side effects.