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: The intrinsic lesions of the brainstem in children lead to a variety of diseases. Although their differentiation is important; however, their diagnosis is difficult via neuroradiological imaging alone. Herein, we report a pediatric case of a developmental venous anomaly (DVA) sited at the brain stem. The patient’s initial head MRI revealed an intrinsic lesion with ill-defined and brush-like contrast enhancements, which was primarily suspected to be a brain tumor. However, the atypical clinical course of the patient and neuroimaging findings such as the rapid exacerbations and remissions in a short time period, and rack of lesion volume effect observed in MRI, refrain us from proceeding with highly invasive examination and initiating any type of treatment including chemotherapy, radiotherapy and ／ or administration of steroids. Consequently, a complete recovery of symptoms and improvement in neuroimaging findings were achieved during the natural course of the disease. Retrospectively, MRI with contrast mater at the initial visit revealed a DVA traversing across the medulla oblongata, suggesting that the this case’s pathophysiology was based on a venous malformation of the medulla oblongata with stagnant outflow and stenosis, causing increased venous pressure and contributing to the clinical symptoms and findings on neuroradiological imaging. In this study, we report this extremely rare case of DVA with a literature review. [ABSTRACT FROM AUTHOR]