Background. The incidence of CNS involvement in Hodgkin lymphoma (HL) is 0.05% and it can occur at the time of initial diagnosis or in a relapsed/refractory disease. Case report. 32-year-old female immunocompetent patient with classical HL (mixed cellularity) in initial clinical stage IIB was treated in Lybia between 2011-2014 (6xABVD, 3x ICE, 4x ASHAP, involved field irradiation of 36Gy on cervical lymph nodes, 2 cycles of Gemcitabine and Navelbine, steroid treatment) without achieving a sustained remission and HL was considered primary refractory. The patient was admitted to our hospital with headache, blurred vision and with disseminated disease in clinical stage IVA (2015): PET/CT confirmed involvement of lymph nodes on both sides of diaphragm, liver, diffuse skeletal and muscle infiltration. MRI of the brain revealed involvement of paranasal sinuses, focal lesions in the right temporal lobe with perifocal oedema and dislocation of the right lateral ventricle. A new biopsy of the cervical lymph node confirmed HL- nodular sclerosis. Cerebrospinal fluid and bone marrow examinations were negative for HL involvement. She was treated with two cycles of brentuximab vedotine and bendamustine (BVB) in combination with dexamethasone. Two cycles of BVB alternated with two cycles of high-dose methotrexate 3,5g/m2 with procarbazine and dexamethasone. She achieved a partial remission according to PET/CT and MRI of the brain after above mentioned treatment. The patient received conditioning regimen BCNU/etoposide/thiothepa followed by autologous stem cell transplantation (ASCT) in February 2016. Neutrophil and platelet engraftment was reported on day+10 after ASCT. Currently, consolidation treatment with brentuximab vedotine is ongoing since day +30 after ASCT. Conclusion. BVB combination is a highly effective salvage chemotherapy for relapsed/refractory systemic Hodgkin lymphoma, and additional effect of bendamustine on CNS involvement in combination with high-dose methotrexate, procarbazine and dexamethasone can be anticipated. Conditioning regimen directed against CNS and systemic HL involvement CNS may further potentiate the effect of salvage chemotherapy. This work was supported by grant awarded by AZV 16-29857A, Ministry of Health in Czech Republic and Research project P 27/2012 awarded by Charles University in Prague, 3rd Faculty of Medicine, Prague, Czech Republic