· Background: Eosinophilic granuloma of the soft tissue, Kimura’s disease, is a benign slow-growing tumor that is manifested clinically by one or more inflammatory nodules involving mainly the face and scalp, but rarely the eye. · Case report: The patient was a 32-year-old male with swelling of the left lower eyelid, marked peripheral blood eosinophilia and increased serum immunogloblin E. MRI revealed swelling of all rectus muscles of the left eye, but no tumor mass. Corticosteroid treatment reduced the swelling of the eyelid, but it recurred after corticosteroid was discontinued. Eight years later the patient returned with a complaint of increased swelling of the left lower eyelid. An elastic, nontender, soft tumor mass was palpable subcutaneously in the left lower eyelid extending into the orbit. MRI revealed a tumor mass in the left orbital space. The parotid gland was also swollen and palpable. Both tumors were resected surgically, and histopathological study revealed prominent proliferation of lymphoid follicles with germinal centers showing interfollicular infiltration by eosinophils. The pathological findings in the parotid gland were similar. The diagnosis was Kimura’s disease. · Conclusion: This patient is unique in that he had no tumor at the first examination, only swelling of the rectus muscles, and a tumor mass appeared many years later. Unilateral swelling of the rectus muscles may be one of the first signs of Kimura’s disease. Not only tumor but also swelling of the rectus muscles limited ocular movement.