The history and confusing terminology regarding reports of inflammatory pseudotumor of the orbit are reviewed. The wide clinical spectrum and variety of associated conditions is emphasized. Twenty-seven patients from the Wills Eye Hospital with clinical records and histopathologic material consistent with orbital pseudotumor were reviewed and an attempt was made to correlate the histopathologic findings with clinical presentation, response to steroids, and final outcome. Adequate follow-up averaging 3.6 years was obtained in 17 cases. Most cases in the series had a favorable outcome, and patients demonstrating the acute and subacute stages of Type II patterns responded to systemic steroids. Repeated and extensive surgical intervention appear to be harmful.