Pineal Tumours account for less than 1 % of all brain Tumours. A proportion of children with bilateral retinoblastoma will develop a pineal Tumours (the so-called trilateral retinoblastoma). Both familial pineoblastoma and familial pinealcytoma can occur but are very rare (Peyster et al. 1986; Gempt et al. 2012). Pineoblastoma has occasionally been reported in association with familial adenomatous polyposis coli. Extragonadal germ cell Tumours may arise in the pineal gland and be associated with Klinefelter syndrome or 46XY gonadal dysgenesis. Pineoblastomas are also seen in carriers of DICER1 mutations (Sabbaghian et al. 2012).