Background: Balamuthia mandrillaris is a rare protozoan pathogen that causes severe central nervous system (CNS) infections in humans. Given the complexity and rarity of these infections, understanding the radiological features is key for early diagnosis and management. This case series aims to elucidate the spectrum of imaging findings in microbiologically confirmed Balamuthia CNS infection cases. Methods: A retrospective study analyzing imaging findings of 20 patients with confirmed Balamuthia CNS infections collected from the hospital’s archives, all of whom had positive CSF cultures and underwent gadolinium-enhanced MRI scans. Results: Patients presented with non-specific symptoms including headaches and seizures. Imaging revealed multiple intra-axial enhancing lesions with surrounding vasogenic edema, some demonstrating ring enhancement and typical imaging features of intracranial abscesses. Cerebritis, hemorrhagic infarcts and necrosis were also noted. Conclusions: CNS infections have a diverse group of causative organisms, including amoebic ones like Balamuthia, and often present with overlapping symptoms, complicating diagnosis. Accurate and timely imaging recognition, combined with CSF analysis, is essential for diagnosing and managing patients promptly, improving overall patients outcome in Balamuthia mandrillaris CNS infections.