In Vietnam, only few cases of cystic echinococcosis have been reported up to now. We report on the first case of cystic echinococcosis involving the central nervous system reported in Vietnam. Our patient, a 14-year-old child, presented with symptoms of headache and progressive tremor in the right hand that had lasted for 1 month and had worsened in the last days before admission. Following the demonstration of a large space occupying brain cyst by computed tomography, a presumptive diagnosis of a brain tumour was made, that was later overruled. Magnetic resonance imaging, a biopsy and serological analysis allowed for the diagnosis of a giant hydatid brain cyst. The patient underwent surgical removal of the cyst and was treated for 6 months post-surgery with albendazole. On follow-up, the clinical symptoms gradually improved, serology became negative and 1.5 years after the surgical intervention the patient was declared to be cured. This case highlights the importance of considering hydatid disease in differential diagnoses, even in regions where Echinococcus infections are considered rare.