Peripheral nerve sheath tumors are common soft tissue neoplasms and their characterization is often challenging. Although the surgical pathology defines some typical entities, some degree of controversy regarding the classification of these tumors still exists. Newer imagistic and histopathological techniques are crucial for their accurate diagnosis and grading. We present an unusual case of median nerve schwannoma in a young patient, discussing the clinical, surgical and pathological elements, including immunohistochemistry.