We present the case of a 14-year-old Caucasian female, with intracranial hemorrhage due to an extensive brainstem cavernous hemangioma, asymptomatic until the date of her admission. Further investigation revealed multiple brain cavernous hemangiomas with two supratentorial hemangiomas demonstrating signs of past bleeding, a cervical vertebral dysplasia (Klippel-Feil type II) and nephrolithiasis, in addition to her history of polycystic ovaries and impaired glucose tolerance, all together presenting a novel clinical combination.We are describing the management of such a patient, according to our current knowledge, inquiring into the possible connections between the conditions detected, as well as, discussing the questions that arise from this rare case.