Hydrocephalus is an excess accumulation of cerebrospinal fluid in or around the brain that can be produced by a broad spectrum of disorders. It can develop at any age and its incidence is increasing, both in infants and adults. Although the standard treatment of hydrocephalus is cerebrospinal fluid shunting, there are certain circumstances in which medical treatment, alone or in combination with shunting, has been suggested as an alternative. This review aims to present and discuss the indications for pharmacological treatment in the medical management of hydrocephalus, and the drugs most frequently used. Carbonic anhydrase inhibitors, loop diuretic agents, osmotic agents and fibrinolytic therapy are discussed. The most suitable drug seems to be acetazolamide, alone or in combination with furosemide. At present, osmotic agents are no longer used in the treatment of hydrocephalus. Fibrinolytic therapy administered directly into the ventricular system may not avoid the need for shunt placement, but may help in the management of hydrocephalus by preventing or reducing the rate of catheter obstruction and accelerating clot resolution.