Spondyloarthritis presents in various and occasionally unusual ways that imitates other diseases. Without forthcoming risk factors, such atypical presentation may elude diagnosis for months. The case presented here of a child, aged 4 years, who is negative for human leukocyte antigen B27 (HLA-B27) and with no family history of HLA-B27 related disease, who developed torticollis with neck pain and lymphadenopathy, highlights the necessity of continually evaluating a diagnosis, especially when treatment fails to produce expected results. Painful torticollis in a child with adenopathy often is infectious in nature or potentially due to Griesel syndrome when persistent. Chronic arthritis of the cervical spine may enter the differential diagnosis when torticollis is persistent, and early recognition and aggressive treatment is necessary to prevent permanent functional impairment.