Abstract: Orbital inflammatory pseudotumor (OIP) refers to benign nonspecific inflammatory conditions of the orbit, which usually occurs in young adults. Single or multiple intraorbital structures  may be involved. Extraorbital extension can also occur. Although the causes of OIP are unknown, it is thought to be associated with autoimmune condition or infection. The clinical manifestation varies according to the anatomic involvement and the degree of inflammation progress. Common clinical manifestations include ptosis, diplopia, poor vision, eyeball swelling, pain, proptosis, etc. Both computed tomography (CT) and magnetic resonance imaging (MRI) are frequently used to investigate OIP. OIP is an exclusion diagnosis based on a combination of clinical and radiographic findings. The mainstay initial therapy for OIP is oral corticosteroids and immunomodulatory agents. Lowdose radiotherapy has been successful in many cases of recurrent myositis. Limited inflammatory pseudotumor can be treated surgically.   (Int Rev Ophthalmol, 2014, 38:   410-414, 419)