BackgroundAIDS existing with cerebral sparganosis is rare. Due to atypical symptoms of sparganosis, diagnosis and treatment are difficult and the misdiagnosis rate is very high. This paper analyzes the clinical data of the first case of AIDS existing with cerebral sparganosis in our hospital and reviews the relevant literature. It discusses the clinical manifestations, imaging features, diagnosis, and treatment of this disease and provides a reference for the treatment of AIDS existing with parasitic encephalopathy (cerebral sparganosis). Cerebral sparganosis has been reported worldwide especially in Asian countries. To date, this is the first reported case of sparganosis in HIV/AIDS patient.Case presentationA 34-year-old newly diagnosed HIV Chinese woman from Guanxi province presented at our hospital with a history of recurrent seizures for 5 years. The initial diagnosis was epilepsy but she was later diagnosed with cerebral sparganosis at our facility after thorough history, examination and investigations. Considering her immune status, she was treated with high dose praziquantel instead of surgery with significant improvement. She also received Zidovudine + Lamivudine + Nevirapine as the first-line antiretroviral treatment due to its high permeability across the blood-brain barrier.ConclusionClinicians should pay attention to this rare entity in cases of HIV/AIDS-related encephalopathies which can be easily misdiagnosed as toxoplasmosis or cerebral tuberculosis. Thorough epidemiological history, clinical presentations, serologic and radiological tests are essential to reach the correct diagnosis and rule out other infections. Although the standard treatment for cerebral sparganosis is surgery, we opted for pharmacological treatment in consideration of immunological complications.This case calls for more studies for standard treatment in HIV patients with cerebral sparganosis and requires clinicians to consider this rare entity when dealing with HIV related encephalopathies.