Cervical intramedullary schwannomas are extraordinarily rare. Gross total resection is the best therapeutic option for these types of tumors. Although rare, intramedullary schwannomas should be considered as a differential diagnosis of intramedullary lesions since a good prognosis can be guaranteed to the majority of these patients. We present a case of a cervical intramedullary schwannoma surgically treated in a 19-year-old male patient who initially presented with motor neuron disease.