Spinal tuberculosis constitutes 50% of all musculoskeletal tuberculosis. However, literature regarding congenital spinal tuberculosis is very scanty. Congenital spinal tuberculosis was diagnosed in a two-month-old child on the basis of age at presentation (gibbus since three weeks of age), hepatomegaly, raised ESR, radiological destruction of D10–D11 vertebrae, asymptomatic maternal endometrial tuberculosis and tuberculous histopathology from CT guided biopsy specimen from D10 vertebra. Both child and mother were treated by antitubercular treatment. Child improved symptomatically, gibbus became less prominent and ESR became normal at the end of one year of treatment. Patient had no recurrence during last two years of follow-up. This is the first case of congenital tuberculosis of spine with a documented source of infection from asymptomatic maternal endometrial tuberculosis. CT guided core biopsy from vertebra/aspiration from the paravertebral abscess help in early diagnosis and treatment to reduce neurological morbidity and mortality. Endometrial biopsy help in establishing the diagnosis of congenital tuberculosis and adequate antitubercular treatment in such cases may provide protection to fetus in subsequent pregnancy.