Abstract: Objective: To explore the clinical manifestations, histopathological features, and imaging characteristics of atypical malformation/rhabdoid tumor (atypical teratoid/rhabdoid tumor) of the central nervous system, to improve the ability of imaging diagnosis and differential diagnosis of this disease. Methods: Clinical realization and imaging findings of an atypical abnormal pattern/rhabdoid were reviewed. Results: Computed tomography revealed a slightly dense right frontal lobe and the lesion had a significant occupancy effect with a visible band of nodular edema around it. The bone window showed no adjacent bone destruction. Magnetic resonance imaging (MRI) revealed the lesion as low signal on T1 and T2-weighted imaging, and fluid attenuation and inversion recovery had a slightly higher signal. A diffusion-weighted imaging sequence showed high signal, uniform lesion signal, and lobulation. MRI enhancement showed less uniform enhancement, edge line enhancement, and a broad base attached to the brain stem. Results: Atypical teratoid / rhabdoid tumors of the central nervous system are rare, with diverse clinical manifestations, lack of specificity of imaging findings, and histopathological examination is still required for diagnosis.