Namaqualand hip dysplasia (NHD) is an inherited skeletal disorder that has been identified in 45 persons in five generations of a large family in South Africa. Involvement of the femoral capital epiphyses, which was present in all cases, led to significant orthopedic complications. Hip pain developed between three and 20 years of age and heralded the onset and gradual progression of premature degenerative arthropathy. The patients who were examined were all over 35 years of age; four had been treated by total hip arthroplasty. For appropriate and effective management of this uncommon but well-defined syndromic entity, an early and specific diagnosis is essential.