OBJECTIVE AND IMPORTANCE: This case demonstrates an unusual associatio
n between arteriovenous malformations and an intracavernous anterior c
erebral artery origin. To the best of our knowledge, this relationship
has not been previously described. Identification and understanding o
f this relationship are important in pre-embolization and surgical pla
nning and in offering some insight into neurovascular development. CLI
NICAL PRESENTATION: The patient presented with severe recurring headac
hes and an otherwise nonfocal neurological examination. He maintained
a stable neurological course throughout evaluation and therapy. INTERV
ENTION: The patient underwent endovascular embolization of the arterio
venous malformations without consequence. He was then scheduled for ra
diosurgical treatment planning. CONCLUSION: This case demonstrates an
unusual neurovascular anomaly with associated arteriovenous malformati
ons. To the best of our knowledge, this is the first reported case of
such an association. An understanding of anomalous angioarchitecture a
nd neurovascular development is essential for prudent endovascular and
surgical planning.