Abstract: The authors reported a case of primary diffuse large B-cell lymphoma（DLBCL） of the skull with ¹⁸F-FDG PET/CT images. Primary skull lymphoma is clinically rare and easily misdiagnosed due to lack of specific clinical manifestations and imaging features. In this article, the clinical manifestations, laboratory examination and imaging features of this case were described in order to improve diagnostic accuracy among radiologists for this rare disease.