Objective: To investigate the clinicopathologic features, diagnosis and differential diagnosis of subependymal giant cell astrocytoma (SEGA). Methods: Eight cases of SEGA were studied by clinicopathology, imaging, and immunohistochemistry, all cases were followed up. Results: The age of patients ranged from 10 months to 37 years (mean 15.5 years), 3 males and 5 females. The tumors mainly occurred in the lateral ventricle. Most patients present with headache and dizziness with nausea and vomiting (5 cases), followed by epilepsy (2 cases), 1 patient showed left eye visual acuity, hydrocephalus occurred in 4 cases with different degrees. The operation was performed in all cases, total resection in 6 cases, subtotal resection in 2 cases, and one of that accepted radiotherapy after subtotal resection. All of the 8 cases were followed-up for 4–66 months. 2 cases were alive with disease after recurrence and a second subtotal resection. 6 cases were disease-free. On CT, SEGA presented as solid or cystic masses located in the walls of the lateral ventricle (Monro hole), often with irregular or nodular calcification, clear or lobulated boundary. Sometimes, unilateral or bilateral ventricular dilatation and hydrops. On MRI, the tumors are isointense, or slightly hypointense on T1-weighted images, and hyperintense on T2-weighted images, with contrast enhancement in solid area, while not in cystic area. Histologically, the tumor cells presented as ground-glass like polygonal cells with abundant cytoplasm to spindle cells trapped in fibrous stroma. The tumor cells are sheet like, clustered or perivascular palisade like structures. Often accompanied by calcification, rich dilated capillaries, perivascular lymphocyte sheath, pleomorphic nucleus or multinucleated cells. Immunohistochemically, all of the tumors were positive for GFAP, S-100, Nestin, Vimentin, and negative for Neu-N, NF, Olig-2. Two cases focally expressed Syn. The Ki67 labeling index were 2%–6%. Conclusion: SEGA is a rarely benign tumor of the central nervous system, typically occurred in the wall of the lateral ventricles, WHO grade I. The histomorphology is diverse. Differential diagnosis contains rhabdoid meningioma, gemistocytic astrocytoma, ganglioglioma, glioblastoma, ependymoma. Patients with total resection of the tumor have a good prognosis, while with subtotal resection may need biological targeted therapy.