Background Ganglioglioma is mostly found in cerebral parenchymal, and it is rarely located in the ventricular system. To date, ganglioglioma within the ventricular system has been reported in only 10 cases. Its prognosis and relationship with complicating hydrocephalus are unclear. Methods A total of 7 cases with intraventricular ganglioglioma diagnosed by the surgical pathology examination from June 2004 to April 2011 in our center were retrospectively analyzed. The clinical data were collected from the clinical medical records, and the tumor site, size and basement of tumor were analyzed. Follow up was performed to obtain the clinical outcomes. Results The 7 cases included 5 males and 2 females, with disease onset at 23.6 +/- 14.9 years old. Epilepsy as the initial symptom was observed in 1 case. Reduced hearing, dizziness and weakness of both lower limbs were found in 1 case. Intracranial hypertension were detected in 5 cases, including 1 case complicating by decreased visual acuity. Tumors were located in the lateral ventricle in 5 cases, while 2 cases in the third ventricle. Hydrocephalus was observed in 5 cases, including 2 cases with severe hydrocephalus, and both underwent ventriculoperitoneal shunting. Total resection of tumors was performed in 5 cases, and 2 cases underwent gross total resection. The mean duration of follow-up was 28.7 months (8-90 months). Intracranial hypertension in all cases disappeared. Even radiotherapy post-surgery, one case with GTR relapsed 1 year later. However, the other 6 cases didn't relapse. Conclusions Ganglioglioma in ventricular system is extremely rare, mainly with the symptoms of intracranial hypertension or seizure. The degree of hydrocephalus is closely related to the site of tumor's basement. The prognosis is good after total resection. The patients with GTR should be followed-up.