BACKGROUND:Hereditary multiple osteochondromas (hereditary multiple exostoses, HME) is a rare genetic disease characterized by the development of benign osteocartilaginous tumors that may cause severe limb deformities and early onset osteoarthritis. Total knee arthroplasty (TKA) is the method of choice for the treatment of advanced gonarthrosis, however the surgical management with coexisting severe axial limb deformity remains unclear. CASE PRESENTATION:65-year-old man with HME and extra-articular multi-axial limb deformity was admitted to the orthopedic department due to chronic knee pain and limited range of motion caused by secondary osteoarthritis. Regarding to the clinical and radiological examinations, after preoperative planning he was qualified to a one-stage TKA combined with tibial shaft osteotomy (TSO). In a one year follow-up full bone union was confirmed with no signs of implant loosening or prosthesis displacement. Patient was very satisfied, did not report any joint pain and has sufficient range of motion without knee instability. CONCLUSION:The improvement of mechanical axis during TKA is a crucial factor for achieve operative success and long implant survival. Despite the higher risk of complication rate in comparison to two-stage treatment, one-stage TKA with simultaneous TSO should be a considerable method for patients with osteoarthritis and multiaxial limb deformities. This method can give a good clinical and functional outcomes, however should be performed subsequently to careful preoperative planning and proper patient qualification.