OBJECTIVE: We present a case of multiple myxoma-related intracranial aneurysms and reviewed the recent relevant literature to investigate their natural course and develop a reasonable treatment algorithm. METHODS: We have reported a new case of multiple myxoma-related intracranial aneurysms and performed a search of current studies (2001 to the present) in PubMed. The keywords used were as follows (single words or combinations): neoplastic aneurysm, oncotic aneurysm, aneurysm, and myxoma. Only patients with multiple aneurysms resulting from cardiac myxoma and with adequate follow-up information pertinent to the analysis were included. RESULTS: A total of 41 patients, including our present patient, were studied. Of the 41 patients, 28 had received conservative treatment as the primary choice. Most lesions (n = 22) were stable during follow-up, except for 3 in which aneurysm evolution was observed. A total of 8 patients had undergone microsurgery, including aneurysm resection in 3, aneurysm clipping in 2, clipping followed by resection in 2, and aneurysm trapping in 1. Endovascular treatment was performed in 2 patients. Radiation therapy was used in 1 patient, and the parent artery proved to be occluded at the 1-year follow-up examination. A combination of chemotherapy and microsurgery was used in 1 patient. All the cases that had been managed aggressively were stable during further follow-up. CONCLUSION: The prognosis was good for most patients with multiple myxoma-related intracranial aneurysms, and most myxoma-related aneurysms were stable. Conservative treatment and routine follow-up are recommended for most patients. However, for patients with evolving or ruptured aneurysms, invasive treatment, including open surgery and endovascular treatment, should be considered.