A number of conditions have been found to be associated with the HLA-B27 locus including ankylosing spondylitis, psoriatic spondylitis, Reiter's syndrome, juvenile arthritis, and others. An unusual clinical syndrome of "sausage-like" toe, or dactylitis, associated with an oligoarthritis involving the lower extremities has been reported in adults, as has HLA-B27 associated spondyloarthritis and enthesopathy in children. We describe 3 children with dactylitis of the toe who carry HLA-B27. Their course and treatment are presented, and the importance of establishing this diagnostic entity is discussed.