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Purpose: To observe the clinical findings and response to treatment in patients with a diagnosis of idiopathic orbital inflammatory pseudotumor.Methods: 209 idiopathic orbital inflammatory pseudotumor cases seen between Jan 1,1978 and Dec 31, 1999 in our hospital were evaluated retrospectively.Results: Of the 209 cases, 118 were male and 91 were female; there were 90 in the right eye, 81 left eye and 38 both eyes. Patients ageranged from 4 to 80 years (mean 44.4). Proptosis (66%), palpable mass (65%), swollen eyelid (55%), increased orbital pressure (55%) and motility restriction (48%) were the five most common presenting signs. According to radiologic and surgical findings, focal mass within orbit was the most frequent subtype(43% ), followed by lacrimal inflammatory pseudotumor (32%), diffuse orbital inflammation (10%), myositis (8%) . Perineuritis (2%),periscleritis(2%), acute inflammation (2%) and eyelid pseudotumor (1%) were rare clinical findings. The response to treatment (with a mean follow-up of 1.5 years)showed that the cure rate was 40% and the effective rate 97% after combined management of surgical resection, systemic steroid and local low dose radiotherapy.Conclusions: Although recurrence of IOIP is common, the success rate of treatment for this group of patients is high.