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Primary intraventricular haemorrhage (PIVH) is an uncommon type of intracerebral haemorrhage, accounting for only 0.31% of all strokes and 3.1% of all intracerebral haemorrhages. Due to the low incidence of PIVH, little is known about its clinical characteristics, risk factors, aetiologies, prognosis and treatment. Acute hydrocephalus is common and is associated with a poor prognosis. External ventricular drainage (EVD) could promptly reduce intracranial pressure by diverting cerebrospinal fluid and intraventricular blood; however, the incidence of complications such as central nervous system infection, catheter occlusion and rebleeding are relatively common. Despite being an invasive procedure, frontal minicraniotomy is an available therapeutic option to avoid complications of EVD. The authors report a case of a PIVH managed with frontal minicraniotomy and perform a literature review about epidemiological data, clinical features and treatment of PIVH., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)