Intramedullary schwannomas are benign tumors that arise from clusters of Schwann cells embedded in the medullary parenchyma. They are very rare and account for 0.3 to 1.5 % of all spinal schwannomas. It is not known for certain why this happens, and several theories have been put forward. They do not exhibit any characteristic clinical picture, being pain the most frequent symptom. Imaging is also not specific and the correct diagnosis is attained most of the times after pathological examination. The goal of treatment must be complete removal, which is curative. We are presenting two patients in whom an intramedullary tumor was removed. The true nature of the lesions was suspected during the operation, but only after pathological examination was confirmed. A review of the literature has been undertaken, with special attention to the theories about the origin of these uncommon tumors.