Tufted angioma is a benign vascular tumor in which immature endothelial and pericyte cells and lymphatic vascular endothelium grow. It manifests as a flat, painful erythema that gradually expands mainly on the trunk and extremities. Although tufted angiomas can also occur in other areas of the body and may be more locally invasive, they rarely occur intracranially. A 63-year-old man underwent magnetic resonance imaging (MRI) for a brain check-up 8 years before his visit to our institute, which detected a mass lesion with surrounding cerebral edema in the left frontal lobe. The patient was followed up with annual MRI analysis, which indicated slow tumor growth and gradual development of peritumoral edema. The tumor was treated by gross-total resection. Histological analysis showed a slightly dilated microvascular core surrounded by many capillary aggregates in the brain parenchyma. Immunohistochemical findings indicated that the vascular endothelial cells were positive for CD34 and Brahma-related gene-1 and were surrounded by smooth muscle actin-positive pericytes. These findings were consistent with tufted angioma. Intracranial tufted angioma is uncommon, but it should be considered in the differential diagnosis for intracranial tumorous lesions. Long-term follow-up is necessary to unravel the natural history of the disease.