Children presenting with torticollis demand thorough investigation. Atlantoaxial subluxation and rotatory fixation should be included in the differential diagnosis even in the absence of neurologic findings. We describe a 9-year-old boy with this condition associated with HLA-B27 positive seronegative spondyloarthropathy that went undiagnosed for 11 weeks. No other radiographic changes were seen, including no erosions. No neurologic findings were present. The child was treated with Halter head traction, which successfully reduced the atlantoaxial joint. This was followed by posterior C1-C2 fusion secondary to instability. Rheumatologists should be aware of this condition when examining a child presenting with torticollis.