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The primary lymphoma of the skull is very rare. We describe a case of a 48-year-old woman with a palpable, soft, painless swelling of the soft tissues of the right frontal region, in absence of neurological symptoms. The ultrasonography showed the presence of solid, very vascularized, subcutaneous, and intra-thecal tissue and bone erosion of the internal and external cranial theca. A CT scan of the skull, thorax, and abdomen was performed to exclude further manifestations of the disease and an MRI examination of the brain to exclude involvement of the intracranial structures. CT and MRI confirmed the presence of the intra-thecal and extra-thecal mass that infiltrated the external and internal cranial theca, the scalp, and the epidural region without infiltration of the cerebral cortex. The patient underwent mass removal surgery and histological analysis, which allowed the diagnosis of non-Hodgkin B cell lymphoma.