Heberden nodes affect mainly middle-aged women. Inheritance is autosomal dominant in female and autosomal recessive in male patients. We report the case of a young man who presented already with 12 years of age with pain in the distal finger joints. There were no other clinical or serological signs for other rheumatoid diseases, like psoriatic or rheumatoid arthritis. Radiologic findings were consistent with Heberden's osteoarthritis of the finger joints. The joint changes remained clinically and radiologically stable during a time period of more than 15 years. The HLA typing revealed the haplotype HLA A1, B8 and DR4, in accordance with former studies which reported a higher frequency of HLA A1, B8 in families with primary osteoarthritis (early onset osteoarthritis of the large joints in combination with Heberden nodes).