Behçet's disease (BD) is a systemic vasculitis commonly accompanied by recurrent mucosal ulceration and other systemic manifestations, but rarely by myositis. Focal eosinophilic myositis is the most limited idiopathic eosinophilic myopathy characterized by peripheral blood eosinophilia and/or eosinophilic muscle infiltration. Clinical manifestations include myalgia, muscle weakness, and cutaneous lesions, such as subcutaneous induration and erythema. Given that BD can mimic deep vein thrombosis or pseudotumor, muscle biopsy should be performed to enhance the accuracy of diagnosis. Microscopic examination reveals extensive infiltration of eosinophils and mononuclear cells into muscle, myofiber necrosis, and regeneration. To the best of our knowledge, there have not been any published reports on MEDLINE regarding focal eosinophilic myositis associated with BD. Here, we presented a case of focal eosinophilic myositis associated with intestinal BD in a 23-year-old man who suffered from a large ulcer in the terminal ileum.