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Objective:Terminal myelocystocele(TMC) is a closed form of neural tube defect, presenting a large, fully epithelialized, cystic lumbosacral mass containing fat, cerebrospinal fluid and neural tissue. This report demonstrates the clinical manifestation, surgical management, and the outcome of TMC patients. Methods:The medical records of the six patients(male/female=3/3) with surgically and histologically proven TMC, registered between 1991 to 2001, were retrospectively reviewed. The initial presentation, features of the lumbosacral mass, urodynamic study, motor function of the lower extremity, the age at the repair and postoperative status were investigated. Results:Initial symptoms and signs were low back mass, urinary/fecal incontinence, and lower limb deformity. One patient was associated with cloacal exstrophy, one patient had imperforated anus, and three patients had foot anomalies. All patients had urological problems on urodynamic study. Neurosurgical correction of the TMC was undertaken at the median age of three months. The mean follow-up period was 71 months(range:9-136 months). Four patients voided with clean intermittent catheterization postoperatively. One patient underwent augmentation ileocystoplasty and Malone operation. One patient had orthopedic operation. Conclusion:Understanding about the characteristic features of TMC and its embryogenesis is necessary for the diagnosis of this entity. For the management of the TMC, early prophylactic surgical intervention is recommended to prevent secondary deterioration in neurological function. Postoperatively all patients must be followed-up long-termly on urological function and lower extremity status. Key words:Terminal myelocystocele;Occult spinal dysraphism;Lumbosacral mass.