Background Total knee arthroplasty (TKA) in patients with skeletal dysplasias is particularly challenging as a result of the anatomic variances and substantial bony deformities. Little has been written regarding technical considerations that should be made when performing TKA in skeletal dysplasia. Questions/purposes We describe special operative considerations that must be made when performing TKA on patients with skeletal dysplasia, including implant selection and ligamentous balancing. Patients and Methods We retrospectively reviewed 12 TKAs in eight patients with varying degrees of deformity (ranging from 30°of varus to 45°of valgus) secondary to three types of skeletal dysplasias: multiple hereditary exostosis, achondroplasia, and osteogenesis imperfecta. Clinical notes, operative records, and radiographic data were reviewed. Minimum followup was 1 year (average, 4 years; range, 1-10 years).