A large intracranial lipoma, located on the superior medullary velum Is reported. Histologically, the tumor consisted of adult adipose tissue. The right trochlear nerve was involved within the tumor, and the scattered nests of Schwann cells and some larger vessels were also present. Lipoma of the dorsal region of the mid‐brain is rare and has not been reported from Japan. Schwann cell proliferation in the lipoma is another unusual feature in this case. The histogenesis of this tumor is discussed, and the literature on lipomas of the dorsal region of the mid‐brain is reviewed.