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We present a case report of a patient with severe valgus deformity of the right knee due to multiple hereditary exostoses (MHEs) treated with total knee arthroplasty (TKA). The surgical management of MHE affecting the knee encompasses exostoses resection, joint deformity rectification, and limb-length discrepancy alignment. On rare occasions, distraction osteogenesis and TKA have been used to correct valgus deformities of the knee. TKA in MHE patients with knee involvement has only been described in 6 cases. Several considerations, such as extensive knowledge of frequently occurring skeletal aberrations, are required to successfully correct the deformities associated with MHE via TKA. This report describes a case of severe valgus knee deformity with a rotational component in MHE managed with TKA, the surgical technique, and future recommendations. Keywords: Exostoses, Knee, Arthroplasty, Replacement, Valgus, Total