A rare case of hypothalamic hamartoma with unusual radiologic and histopathological features is described, possibly the first of its type in English literature. A 1.5-year-old female child presented with precocious puberty. MR scan of the brain revealed a pedunculated hypothalamic mass, most of which was isointense with normal brain on T1- and T2-weighted images. However, a sizeable component of the lesion was hyperintense on T1-weighted images, suggestive of adipose tissue. Microscopically, the lesion was a hamartoma composed of an admixture of neuroectodermal elements, namely glial cells, neurons, and nerve bundles along with mesenchymal elements in the form of fibroadipose tissue.