Objective To study the advantage and major features of minimally invasive management of neoplasms in the fourth ventricle. Methods Twenty-eight cases of the fourth ventricle neoplasms underwent craniotomy through the cerebellomedullary fissure approach. Postoperative treatment and follow-up information were studied. Results Of the 28 studied cases, total resection was achieved in 23, and subtotal resection in 3. No approach-related complications such as cerebellar mutism, injury of posterior cranial nerves and hemiplegia occured. Conclusion The minimally invasive management of neoplasms of the fourth ventricle by cerebellomedullary fissure approach is safe and effective.