The hypothalamus is susceptible to involvement by a variety of processes, including developmental abnormalities, primary tumors of the central nervous system (CNS), vascular tumors, systemic tumors affecting the CNS, and inflammatory and granulomatous diseases. The hypothalamus may also be involved by lesions arising from surrounding structures such as the pituitary gland. Magnetic resonance (MR) imaging is the modality of choice for evaluating the anatomy and pathologic conditions of the hypothalamus. The MR imaging differential diagnosis depends on accurate anatomic localization and tissue characterization of hypothalamic lesions through the recognition of their signal intensity and contrast material enhancement patterns. Diffusion-weighted imaging and proton MR spectroscopy can be helpful in differentiating among various types of hypothalamic lesions. Key MR imaging features, in addition to the patient's age and clinical findings at presentation, may be helpful in developing the differential diagnosis for lesions involving the hypothalamic region.