A 47-year-old man presented with a subarachnoid hemorrhage (SAH) and right cerebellar hematoma was referred for evaluation. Cerebral angiography revealed a distal anterior inferior cerebellar artery (AICA) aneurysm associated with an arteriovenous malformation (AVM). Successful obliteration and complete removal of the aneurysm and AVM were obtained using transcortical approach under the guidance of neuronavigation system. The association of a peripheral AICA aneurysm and a cerebellar AVM by the same artery is unique. The reported cases of conventional surgery for this disease complex are not common and their results are variable. Less invasive surgery using image-guided neuronavigation system would be helpful and feasible for a peripheral aneurysm combining an AVM of the posterior fossa in selective cases