We present the first reported case of a mother and daughter with unilateral Kienbock’s disease, along with a radiographic and genetic study of the case. The radiographic study demonstrated ulnar minus and flattened radius in both patients. The karyotype of both affected individuals (mother and daughter) was normal 46XX (peripheral lymphocytes). Since the chromosomal analysis was normal, we could not speculate with regard to genetic etiology, or suggest further molecular investigation. If more familial cases become available, it would be possible to investigate this in the future.