Craniovertebral junction tumors represent a complex pathology carrying a high risk of injuring the vertebral artery and the lower cranial nerves. Dumbbell C1- C2 schannomas are very rare tumors in this location. We present a case of a 66 years old male accepted for left laterocervical localized pain, headache and vertigo, with a large C1 dumbbell schwannoma extending in lateral over the C1 arch and displacing the C3 segment of the vertebral artery superiorly and anteriorly. Complete removal of the tumor was achieved using a far lateral approach. The approach is discussed with focus on the vertebral artery anatomy as the approach should give enough space to gain control of the artery without creating instability. Safe removal of C1 nerve root schwanomas can be achieved even if they compress and displace the vertebral artery by entering a fibrous tissue plane between the tumor and the vertebral artery.