Instability of atlantoaxial joint secondary to Tuberculosis or Myeloma is a rare entity. Biopsy along with stabilisation is a described operative procedure. We presented a case of 47 years old male with C1-2 affection treated with occipitocervical stabilisation and biopsy proved as tuberculosis. Antituberculosis treatment was started and clinical improvement was apparent in 5 months. Aggressive progression of diseases after 5 months prompted us to investigate further. Reviewing the biopsy slides with repeat transoral biopsy confirmed features of myeloma which finally responded to chemotherapy. Patient improved significantly (neck disability index = 24%) at the follow-up of 3 years. Hence, coexistence of both tuberculosis and myeloma very rare but should be acknowledged