Primary Non-Hodgkin's Lymphoma of the cranial scalp and skull vault is a rare disease. We are describing a case of the same in a 50-year-old man. He was presented with a diffuse swelling in the left side scalp since 4 months of duration and progressively enlarging in size. On local Examination of the scalp, there was a diffuse swelling in the left parietal and occipital region of scalp. Imaging showed diffuse infiltration of the skull vault with extracranial soft tissue masses. Further investigations with CT scan chest, abdomen, and pelvis did not reveal any other evidence of systemic lymphoma. Biopsy of one of the scalp masses showed a small to intermediate cell B-cell lymphoma. Other nine previously reported cases of primary skull vault lymphoma were reviewed.