Cavernous malformations are low-flow vascular malformations that are histologically characterized by the lack of mural elements of mature vascular structures and intervening parenchymal neural tissue. They occur in sporadic and familial forms. Familial cases are associated with a high frequency of multiple lesions, which are less frequently associated with sporadic cases. They represent 10–20% of all central nervous system (CNS) vascular malformations. A supratentorial location of lesions has been reported to be more frequent than an infratentorial location. Hypothalamic location of cavernoma is extremely rare. We present a case of 40-year-old female who presented with progressive left lateral homonymous hemianopsia. Magnetic resonance imaging (MRI) is pivotal in the management of this condition and it is more sensitive than computed tomography (CT) for the detection of cavernomas. We describe the imaging characteristics of the lesion in such a rare location, highlighting the role of MRI which guarantees a higher anatomic ant contrast resolution. We also suggest some clues for differential diagnosis.