Objective To our knowledge, there have not been any reported cases of a meningioma of the craniocervical region presenting solely with syncope as its initial symptom. Only 1 case of meningioma presenting with syncope has been published, but it was associated with hydrocephalus. We report 2 cases of syncope caused by a craniocervical junction meningioma, with syncope being the sole presenting symptom and without hydrocephalus. We discuss the possible pathophysiology, as well as the clinical relevance of this type of presentation. Methods We reviewed the charts, operative details, and imagery of 2 cases of meningioma in the region of the craniocervical junction, with syncope as their sole presenting feature. We also reviewed the literature. Results In 1 case the syncope occurred spontaneously. In the other, it occurred during a Valsalva maneuver. Both meningiomas were surgically removed via a retromastoid approach. There was no recurrence of syncope following surgery. Following a literature review, we found 1 case of posterior fossa meningioma presenting with syncope, but hydrocephalus was also present. Conclusion Syncope can be the sole manifestation of a meningioma of the craniocervical junction. Such syncopes are a consequence of transient dysfunction of the autonomous pathways in the medulla and/or of the medulla's output. In the absence of other causes of syncope, a meningioma in this region, even in the absence of hydrocephalus, should not be considered as fortuitous, but rather as the actual cause of syncope. Recognizing this possibility offers the potential for proper diagnosis and appropriate treatment of the syncope.
