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Schwannomas in the head and neck are a relatively common condition. However, intramuscular schwannomas in the craniocervical region are extremely rare. The preoperative consideration of schwannomas is very important to preserve adjacent circulation and nerve function during the surgery in this area. Moreover, the treatment and preoperative evaluation of this condition is not firmly established in the past literatures. The authors successfully managed the intramuscular schwannoma in the craniocervical region, and provide the differential diagnoses with appropriate treatments.