Cerebral malakoplakia is a very rare chronic inflammatory disease. We herein report the case of a 49-year-old female who presented with a slowly progressive speech disturbance and right hemiparesis. Computed tomography and magnetic resonance imaging showed irregular enhanced mass lesions with numerous scattered areas of calcification in the left insula, thalamus and basal ganglia. Histopathologically, the biopsy specimen showed basophilic laminated inclusion bodies and intracellular and extracellular calculospherules, usually with a typical targetoid appearance (Michaelis–Gutmann bodies). Treatment with antibiotics, bethanechol and ascorbic acid improved her symptoms in association with a decrease in the abnormal calcification and enhancement. The cerebral malakoplakia mimicked a brain tumor in terms of the patient's clinical course and neuroradiological image findings; however, it was successfully cured with medical treatment. This case provides evidence that the pathogenesis of cerebral malakoplakia is deeply tied to bacterial infection and that medical treatment is effective in cases of this disease.