The tumors of middle ear are extremely various.
This variety raises difficult problems of differential diagnosis because of the dissimilar evolution of these tumors. The clinical exam under these circumstances must be completed with the paraclinical exam and especially with the anatomicopathological exam for having an exact diagnosis.
It is represented the case of a tumor of the retrotympanic space which raised difficult problems of differential diagnosis.
The anatomicopathological exam of the surgical piece established the diagnosis of Jacobson nerve schwannoma.
At the control realized after 1 year from the intervention it was not noticed the presence of the relapse.
We considered interesting the presentation of this case because of the variety of this type of tumor and because of the problems of diagnosis and treatment that it raised.