OBJECT: The superior medullary velum (SMV) is a thin lamina of white matter located between the superior cerebellar peduncles horizontally and between the midbrain and cerebellum vertically. The SMV has not previously been described as the primary location of a posterior fossa tumor, although it can be secondarily invaded by a tumor from the cerebellum or quadrigeminal plate. This paper aims to define clinical and radiological features of tumors primarily arising from the SMV during childhood. METHODS: The authors observed 6 infants and children harboring neoplasms of the SMV who were treated at Ann & Robert Lurie Children's Hospital of Chicago (formerly Children's Memorial Hospital) in Chicago, Illinois. Pathological diagnosis of the neoplasms was an atypical teratoid/rhabdoid tumor (ATRT) in 5 patients, and a juvenile pilocytic astrocytoma (JPA) in the remaining child. The tumors were diagnosed during infancy in all patients, with ages ranging from 3 months to 10 months, except for the patient with a JPA (diagnosed at 5 years old). All patients presented with signs and symptoms of increased intracranial pressure due to obstructive hydrocephalus. RESULTS: Characteristic MRI features were noted, consistent with a mass in both the fourth ventricle and the cerebellomesencephalic fissure and quadrigeminal cistern, resulting in the circumferential displacement of the neural structures surrounding the SMV. The tumor was removed effectively in gross-total fashion through the occipital transtentorial approach in all patients. This approach offers a wide exposure of the region. However, all infants with ATRT suffered tumor dissemination and died between 4 and 11 months after diagnosis, in spite of radical resection and oncological treatment. The 1 child with JPA is alive and well 30 months after tumor resection. CONCLUSIONS: To the best of the authors' knowledge, this is the first description in the literature that focuses on tumors originating from the SMV. This entity must be promptly recognized on preoperative radiological studies to carefully plan the subsequent surgical and clinical management.