The clinical manifestations of obstruction of the superior vena cava are discussed, and the infrequent occurrence of this condition in childhood is noted. A case is reported in which obstruction of the superior vena cava caused early enlargement of the head, and the condition was diagnosed when the child was referred for management of hydrocephalus. Increased intracranial venous pressure causes cerebrospinal fluid to be produced in excess of absorption. When this occurs in early childhood, enlargement of the head occurs without manifestations of cerebral dysfunction. The management of this condition and the intracranial complication is discussed.