There is an appreciable mortality associated with BMT in patients with SCID and advanced BCG infection. We present a girl with T-B+ SCID complicated by spina ventosa and disseminated BCG osteitis after receiving a fully matched sibling marrow transplant. Considerable progression characterised by two clinical activations and multiple pleural and perivertebral abscess formations occurred with conventional anti-mycobacterial chemotherapy. She finally recovered with full immune reconstitution after BMT and intensive treatment comprising five conventional and alternative agents that she received for 36 months. No side-effects and/or complications have been seen other than hearing loss.