The human eye can be compromised by a variated spectrum of neoplasms and reactive processes. Here we present a rare case of a primary intraocular inflammatory myofibroblastic tumor (IMT) dependent on the sclera and choroid in a 31-year-old female. The knowledge surrounding IMTs, previously included in the category of inflammatory pseudotumors, has undergone dynamic changes in the past two decades. Here we review the characteristics of these tumors in the human eye and in the surrounding structures, and we describe the recent advances that allow molecular characterization of the neoplastic nature of this entity.