Calvarial tuberculosis is one of the rare infectious diseases that are reported scantily in the literature. This disease could be a rare variety of skeletal tuberculosis affecting the skull. The disease is seen predominantly in developing countries because of malnutrition, poor socio-economic status, immune deficiency disorders, etc. The disease is additionally found occasionally in developed countries because of the migration of individuals. We undertook a systematic review in drafting this review article. We have searched PubMed / MEDLINE, EMBASE, SCOPUS and Google scholar for studies published as original research article, case report, case series and review articles on Calvarial tuberculosis between 2000 and 2024. We also manually searched the reference lists of the included articles to include further studies on this topic. In this review, we briefly discuss the pathogenesis, clinical features, radiological features, diagnosis, and management of calvarial tuberculosis. Knowledge, awareness, and a tall file of doubt are required for early and prompt diagnosis of this scantily reported disease.