BACKGROUND: Cystic dilation of the ventriculus terminalis (CDVT) is a rare anatomic variation that and may become symptomatic. Literature regarding CDVT is limited, and thus the clinical characteristics and management strategy of CDVT are unclear. Here we report 6 cases of CDVT seen in our institution, and provide a review of the current literature. METHODS: The study cohort comprised 6 patients with CDVT seen at Beijing Tiantan Hospital between September 2010 and June 2015. All 6 patients were managed surgically. Data on age, sex, clinical presentation, radiologic features, operative methods, and surgical outcomes were reviewed retrospectively. We evaluated the clinical outcomes and postoperative radiologic changes of these patients and compared them with findings of similar previous reports. RESULTS: All 6 patients were female, with a median age of 50.0 years (interquartile range [IQR], 25.8-54.0 years). Surgical management of cyst-subarachnoid shunting using T-catheter after myelotomy was successfully performed in all patients. During an average follow-up of 41 months, relief of clinical symptoms, especially low-back pain and sciatica, were observed in all 6 patients. No patients experienced symptom recurrence during follow-up, and postoperative magnetic resonance imaging revealed no regrowth of the lesion. Patients with focal neurologic deficits and sphincter disorders also achieved symptom relief from the procedure. CONCLUSIONS: CDVT is an extremely rare cause of conus medullaris syndrome with an unclear treatment strategy. Our successful surgical management of 6 cases through cystic-subarachnoid shunting using a T-catheter for CDVT drainage provides a potential option for treating CDVT.