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Primary Ewings sarcoma of the skull vault is a rare lesion. Metastatic disease from a long bone primary tumor is more commonly seen in the skull.

Patients present with scalp swelling and and tenderness. Depending on the size of the lesion there may be associated neurologic findings. CT scan and MRI delineate the skull vault lesion with associated epidural soft tissue. Excision and pathological are used to differentiate it from other primary skull vault lesions. Surgical removal and radiotherapy and or chemotherapy is the treatment of choice.

The most common site for primary Ewings saccoma is the long bones.
Ewing's sarcoma in the long bones presents with a permeative pattern of medullary destruction. Typically there is a wide transition zone, destruction of cortex, and a periosteal reaction. A soft tissue mass is commonly the presenting sign.