Intraventricular lesions are uncommon, and they can arise from numerous structures around the ventricular system, including the ependyma, septum pellucidum and choroid plexus. Pineal region lesions may arise from the pineal gland parenchymal/supporting cells or glial cells of the midbrain/medial thalamus. Many of these lesions are either found incidentally or present with symptoms of hydrocephalus. Careful assessment of the clinical and radiological features of each case can help to narrow the differential diagnosis in this heterogenous group of tumours.