Background: Manganese encephalopathy is a potential complication of parenteral nutrition. Lack of early recognition leads to unnecessary testing and to continued exposure to manganese. Methods: Case report and review of the literature. Results: We describe the clinical and imaging findings of a patient with manganese encephalopathy in whom the diagnosis was delayed due to lack of recognition of the characteristic imaging findings. Conclusion: Manganese encephalopathy has protean clinical and imaging findings that can easily be overlooked. © 2010 Springer Science+Business Media, LLC.