Our aim is to describe clinicopathological, histochemical, and immunohistochemical findings of one case of inflammatory myofibroblastic tumor of the nasal cavity. A 10-year-old female presented with a short history of nasal obstruction, epistaxis, nasal discharge and headache. Computerized tomography (CT) scans showed a space-occupying lesion in the right nasal cavity. Histological examination of initial biopsy showed fascicles of spindle cells in a mixed inflammatory background with a predominance of plasma cells, typical of inflammatory pseudotumor. The spindle cells were positive for vimentin and actin. The mass was completely excised without any difficulty under generalized anesthesia. Inflammatory myofibroblastic tumor of the nasal cavity is a localized and completely benign lesion. Simple complete excision is curative.