A rare case of giant cerebral varix with venous angioma and intracerebral hemorrhage, surgically treated with good outcome, is reported. Varices and venous angiomas are infrequent in the clinical practice and are usually asymptomatic. Giant venous varices are less rarely associated with vein of Galen aneurysms or high flow arteriovenous shunts, whereas isolated giant varices are exceptional. Association of varix and venous angioma has been reported only in three instances. The radiological diagnosis of the cerebral venous malformation is briefly discussed.