Primary non-Hodgkin lymphoma (NHL) of the skull is extremely rare. The authors report a case in a 19-year-old boy who presented with a progressively increasing scalp mass on background of generalized headache of 6-months duration. Imaging showed multifocal skull vault lesion with intra- and extra-cranial soft tissue masses, causing permeative destruction of underlying skull bone. Further investigation failed to identify any other evidence of systemic lymphoma. Histopathology examination of superficial scalp mass showed low grade non-Hodgkin B cell lymphoma. The precise anatomical location and multifocal nature of the neoplasm occurring in young nonimmunocompromised individual makes this case clinically and radiologically unique.