Multiple myeloma is a hematological neoplasm that frequently affects the spinal column. Less than a fifth of this vertebral involvement corresponds to the cervical spine and cranio-cervical junction. When there is instability or neurological involvement due to compression or deformity, approaches for anterior decompression and occipitocervical stabilization are required. The correct managment of vertebral artery aneurysm associated with occipitocervical arthrodesis requires extensive knowledge of anatomy and pathology. We present a case of a vertebral pseudoaneurysm that occurred late after the resection of a C1-C2 vertebral body multiple myeloma lesion managed with endonasal endoscopic approach and posterior occipitocervical arthrodesis as well as a systematic review of the related literature. The patient recovered well, without major neurological deficits.