Background: The occurrence of familial brain tumours, particularly gliomas, hemangioblastomas in Von Hippel Lindau and other endocrine neoplasia, is well documented in the literature. On the other hand, familial pineal tumours are extremely rare and only a handful of cases have been reported. Methods and Results: Two female siblings presented at ages 12 and 15 with histories of progressive headaches. Neurological examination in each was completely normal. Magnetic Resonance Imaging confirmed the presence of cystic and solid lobulated pineal lesions with mild enhancement, consistent with pineocytoma, in both girls. Follow-up for 15 years in the first sibling and 4 years in the second showed no evolution in radiological or clinical manifestations. No active treatments have been carried out. Conclusion: The occurrence of familial pineal lesions raises the possibility of a close relationship between heredity and oncogenicity, and should be further explored.