Abstract: Objective: This study aimed to investigate the clinical, pathological, and imaging features of primary undifferentiated small round cell sarcoma of the sellar region to improve the ability of imaging and differential diagnosis. Methods: The clinicopathological features and computed tomography (CT) and magnetic resonance imaging (MRI) manifestations of a case with primary undifferentiated small round cell sarcoma of the sellar region confirmed by surgery and pathology were retrospectively analyzed, and the literature was reviewed and summarized. Results: CT showed a node in the right sellar region, reinforcement under-uniform reinforcement and affects adjacent skull; MRI showed homogeneous hypointensity on the T1-weighted image, hyperintense on the T2-weighted image, fluid-attenuated inversion recovery, and diffusion-weighted imaging; and significantly reinforcement with smooth margin on reinforcement scanning. Bone destruction was seen near the posterior clinoid process of the sella turcica. Conclusion: Primary undifferentiated small round cell sarcoma of the sellar region is rare, and its clinical and imaging findings are not significantly specific. Therefore, the diagnosis requires histopathological examination.