Intraventricular hemorrhage (IVH) has been associated with poor prognosis in patients with spontaneous intracerebral hemorrhage. Several factors contribute to the deleterious effects of IVH, including direct mass effects of the ventricular blood clot on ependymal and subependymal brain structures, mechanical and inflammatory impairment of the Pacchioni granulations by blood and its breakdown products, and disturbance of physiological cerebrospinal fluid (CSF) circulation. Acute obstructive hydrocephalus represents a major life-threatening complication of IVH and is usually treated with an external ventricular drainage (EVD). However, treatment with EVD alone is frequently not sufficiently effective due to obstruction of the catheter by blood. In the past two decades, intraventricular fibrinolysis (IVF) has been increasingly used for maintenance of EVD functionality and acceleration of ventricular clot resolution in such patients. Unfortunately, there is no prospective, randomized controlled trial addressing the effect of IVF on clinical outcome. The available data on IVF consist of small retrospective case series, case reports, and a few prospective case-control studies, which are the subject of the present review article. All these studies, when considered in their entirety, suggest that IVF has a positive impact on mortality and functional outcome, and could be considered as a treatment option for selected patients.