Ochronosis is the dark pigmentation of connective tissues in patients with alkaptonuria. The latter is an autosomal recessive deficit of phenylalanine and tyrosine metabolism with various clinical manifestations. We report on a 63-year-old man with familial ochronosis, who presented with a terminal degenerative right hip joint; his older brother had already been successfully treated at our department by total hip replacement. The postoperative course was uneventful and satisfactory for both.