Abstract
Giant perimedullary arteriovenous fistulae (GPAVFs) are extremely rare, particularly cervical GPAVFs
whose incidence has not been tabulated. The occurrence of aneurysm on an artery feeding a GPAVF has
previously not been described. Internal carotid artery aplasia is also very rare (0.01%). The concurrence of
these disorders has previously not been recorded. We report a case of a 5-year-old female with increasing
headaches, who was found to have intraventricular hemorrhage and above anomalies. Coil embolization of
GPAVF and the adjacent aneurysm was attempted. Treatment was complicated by stroke and death.
Embryological and anatomical factors underlying these anomalies as well as, management options are discussed
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