Schwannomas of the nasal cavity and paranasal sinuses are quite rare, with 4% occurring in this location. Most of them are benign and do not recur when totally removed by surgery. It is very important to distinguish between schwannoma and primary benign neurofibroma. Neurofibromas are lesions having the possibility for malignant transformation and recurrence. A case of schwannoma in the nasal cavity is reported, and the diagnostic and therapeutic procedures, as well as recommendations from the literature, are described. The histological and immunohistochemical features are discussed in detail to draw a distinction between schwannoma and neurofibroma. In cases of intranasal and paranasal lesions, the existence of a schwannoma must be considered. Differentiating between schwannoma and neurofibroma is important for estimating the risk of malignant transformation and recurrence.