A 66-year-old man presented with complaints of numbness for the past 5 years and progressive motor weakness of the right leg for the previous 2 months. Magnetic resonance imaging revealed large intra- and extracranial tumors in the frontoparietal region. Physical examination suggested the extracranial lesion was a subcutaneous lipoma, which had been partially resected 60 years before, connected to the intracranial lesion via a defect of the skull. Gross total removal of the tumors was performed. Histological examination showed the intracranial lesion was epidermoid tumor, and connected to the extracranial lipoma by a lipoma bridge. The symptoms improved remarkably following surgery. This case of intracranial epidermoid tumor associated with intra-extracranial lipoma indicates that implantation of skin tissue in childhood carries the risk of epidermoid tumor even after several decades. Neuroimaging screening is recommended to detect the development of any intracranial components.