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Persistent primitive hypoglossal artery (PPHA) is a rare form of persistent embryonic carotid-basilar anastomosis. We present an unusual case of PPHA and an anterior choroidal artery (AChoA) aneurysm associated with Chiari type I malformation. A 45-year-old woman presented with transient dizziness. Magnetic resonance imaging revealed Chiari type I malformation and a left AChoA aneurysm. Digital subtraction angiography incidentally revealed a left PPHA. To the best of our knowledge, this is the first reported case of Chiari malformation i