Perinatal tuberculosis (TB) is a rare infectious disease caused by Mycobacterium tuberculosis. Perinatal TB may be congenital or acquired after birth. Congenital TB is due to transplacental hematogenous spread or ingestion/inhalation of contaminated amniotic fluid or vaginal secretion in the uterus or during childbirth. Neonatal TB may be acquired after birth as a result of ingestion/inhalation of infected droplets or the contamination of skin or mucosal lesions by infected material. The diagnosis of perinatal TB is challenging due to its nonspecific clinical manifestations make it difficult to differentiate from other infections, resulting in a high mortality rate of 40-60%. Therefore, early diagnosis is an essential step in the treatment of perinatal TB. In this paper, we report a case of perinatal TB in a 26-day-old neonate in a remote area of Asmat Regency of South Papua, Indonesia.