Lenticulostriate artery (LSA) aneurysm is relatively rare, and the need for surgical intervention is controversial. Here, we report a case of ruptured LSA aneurysm which was accompanied by familial Mediterranean fever (FMF). A 45-year-old woman with a history of FMF presented with sudden onset of headache and vertigo. Computed tomography revealed hemorrhage in the right caudate nucleus and lateral ventricles. Digital subtraction angiography revealed a fusiform aneurysm (3mm) at the distal site of medial LSA. Observation was selected, and the aneurysm gradually decreased and eventually disappeared on the 105th day from the onset. This was the first report describing an LSA aneurysm associated with FMF. It suggests that such aneurysms could be treated conservatively with close radiological follow-up.