Osteochondritis dissecans is a common disease and is usually accompanied by solitary lesion, especially on the medial femoral condyle. But familial osteochondritis dissecans is rare. A total of 46 families in whom more than one member was affected by osteochondritis dissecans were reported up to 1988 in literature. These families were characterized by multiple articular lesions, autosomal dominant inheritance and short stature. This paper reports one family of familial osteochondritis dissecans. A mother and her three children had some articular symptoms, including in both knees, and short stature. Two of the children were affected by osteochondritis dissecans, whereas the mother showed early degenerative joint disease in both knees. Familial osteochondritis dissecans appears to be caused by disorder of epiphyseal ossification.