Intracranial aneurysm may appear even after the removal of the cardiac myxoma. However, the pathogenesis and treatment of such aneurysm lesions are not clear. The study aimed to explore the clinical and imaging manifestation, hypothetical pathogenesis, and therapy in one case of left atrial myxoma causing multiple intracranial aneurysms. A 14-year-old male displayed a 3-hour history of episodic loss of consciousness and right hemiplegia after a leapfrog-like movement. The myxoma was diagnosed by a combination of clinical examination, leading to the diagnosis of mitral dynamic obstruction with a Grade III mitral diastolic murmur and tumor plop; magnetic resonance imaging, revealing multiple ischemic sites in both semi-oval centers; and transthoracic echocardiography, demonstrating a mitral valve obstruction.The myxoma was removed surgically; however, computed tomography angiography showed multiple intracranial aneurysms in both middle cerebral arteries 18 months after resection of the atrial myxoma. After conservative treatment, the patient had no neurological dysfunction symptoms for 5 years after myxoma resection. His condition is relatively stable. In conclusion, resection of the atrial myxoma may eliminate the early neurological symptoms, but it cannot ensure the nonoccurrence of delayed intracranial aneurysms. The neoplastic process theory was favored for explaining the aneurysm development in this case. According to the specific conditions of the patient, a combination of open surgery, chemotherapy, radiotherapy, and coil embolization is recommended.