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ABSTRACT Introduction Primary angiitis of the central nervous system (PACNS) is a vasculitis confined to the CNS. A small proportion of the lesions may present as a tumor‐like mass, which is rarely seen in children. Case presentation A 5‐year‐old girl was admitted to our hospital because of an intermittent headache. Brain imaging suggested a space‐occupying lesion in the right cerebral hemisphere. The final diagnosis was PACNS with a lymphocytic pattern by stereotactic brain biopsy. Her condition improved after immunotherapy. Conclusion Pediatricians should consider the possibility of PACNS when encountering intracranial tumor‐like lesions. Early diagnosis of tumor‐like PACNS and prompt immunotherapy could improve the long‐term prognosis and avoid surgery.