Tufted angioma is a rare acquired vascular tumor. It is characterized by painful purplish macules that may progress to plaques containing angiomatous papules. The condition is benign; however, it often affects extensive areas of the skin, leading to functional disability of the affected limb if painful. The present report describes a case of a tufted angioma associated with myofascial pain syndrome in which the predisposing element was the presence of this tumor since childhood. Pain at the site of the lesion affected muscle use and led to the onset of the associated syndrome. Complete relief from symptoms was achieved by blocking the trigger points of the affected limb with anesthesia.