Multiple intracranial hydatid cysts are rare and are seen in about 10–15 % of intracranial hydatidosis (Ugur et al. 1998). Ruggiardo, in 1902, was the first to report the perforation of the ventricle by an intracerebral cyst (Valentino 1959). A literature search in PubMed revealed numerous reports of supratentorial intraventricular hydatid cysts (Negovetic et al. 1990; Lunardi et al. 1991; Peker et al. 1991; Cataltepe et al. 1992; Diren et al. 1993; Karadağ et al. 2004; Ciurea et al. 2006; Kamath et al. 2009; Mohindra et al. 2012) and only 9 reports of solitary cysts in the fourth ventricle (Rahimizadeh and Hadadian 1984; Pau et al. 1987; Gelabert Gonzalez et al. 1988; Singounas et al. 1992; Turgut 2001; Bukte et al. 2004; Furtado et al. 2009; Duishanbai et al. 2010; Mohindra et al. 2012). Turgut (2002) described a 13 % incidence of intraventricular hydatidosis based on a meta-analysis of all reported intracranial hydatidosis cases in the pediatric and adolescent group in Turkey. Table 7.1 provides a comprehensive list of all cases with intraventricular hydatidosis in indexed literature to date.