Extraskeletal Ewing’s sarcoma/primitive neuroectodermal tumour (EES/PNET) is a rare disease entity. Scalp EES/PNET has been reported rarely. We report a case of an 11-year-old boy who had painful and rapidly growing subcutaneous nodes over the scalp and neck. The final diagnosis was EES/PNET after biopsy and immunohistochemical assay. The patient underwent surgical excision, chemotherapy and radiotherapy with a dose of 2000 cGy. Now he has been free of disease for two years. Early awareness and treatment of this rare disease, and wide resection followed by chemotherapy and radiotherapy might improve patients’ long-term survival.