Tuberculous spondylitis is not an uncommon disease of the spine. Near one percent of all cases of spinal tuberculosis (TB) involves craniocervical junction. Hypoglossal nerve palsy is not an uncommon neurological finding, but isolated involvement of the hypoglossal nerve is rare and limited to case reports or small case series. Here, we report a case of craniocervical junction tuberculosis presenting with unilateral hypoglossal nerve palsy. Case is a 41-year-old woman with neck and suboccipital pain since one month and unilateral right hypoglossal nerve palsy since one week. All laboratory tests were unremarkable except raised ESR level. Involvement of C1-C2 and hypoglossal canal were demonstrated by CT scan of craniocervical junction. Tissue diagnosis of TB was established by open biopsy of the craniocervical junction.