Intraventricular hemorrhage (IVH) remains a significant cause of morbidity and mortality. Intraventricular alteplase is a recently established therapy, with the clot lysis: Evaluating accelerated resolution of IVH (CLEAR) III trial, being the highest level evidence to date. Historically, as in the CLEAR III trial, there have been notable exclusionary criteria for patients to receive this therapy. The goal of this report is to demonstrate its safe use in a patient with moyamoya. We present a 60-year-old male with severe IVH secondary to moyamoya disease successfully treated with intraventricular alteplase.