A 30-year-old woman with hereditary onycho-osteodysplasia was examined. In addition to the classic tetrad of fingernail and toenail dysplasia, patellar aplasia, iliac horns, and radial head hypoplasia and dislocation, she also had scoliosis, proteinuria, and distinctive bilateral foot anomalies. The foot deformity consisted of a ball-and-socket ankle joint, valgus ankle, forefoot supination, and lateral subluxation at the tarsal-metatarsal joints. The literature showed that the radiographic findings of foot deformity in this dysplasia have never been reported in detail before. Evidence suggests that this distinctive pattern of deformity may be more common than previously appreciated.