Histoplasmosis is a systemic fungal infection caused by inhalation of Histoplasma capsulatum, which is mainly found in bird and bat droppings. Oral manifestation of histoplasmosis may be the only initial manifestation of the disease or associated with chronic disseminated histoplasmosis. The first review of oral histoplasmosis among Malaysian population from 1967 to 1994 (27 years) revealed the occurrence of 37 cases, reported by Ng and Siar in 1996. This current study is the updated overview of oral histoplasmosis cases in Malaysia. The objective of the study was to review and describe clinical and demographic profile of oral histoplasmosis in Malaysia and to correlate histopathological features of oral histoplasmosis with patient's immunity status. We reviewed oral histoplasmosis cases diagnosed in Stomatology Unit, Institute for Medical Research (IMR), Kuala Lumpur from 1995 until 2016. The data was retrieved from the Oral Pathology Information system (OPIS) Stomatology Unit, IMR, which is the largest oral pathology database in Malaysia. Information regarding patients' sociodemographic data, medical illness, clinical presentation, histopathological features, and referring healthcare institutions was extracted from the clinical information which accompanied the biopsy request form. A total of 39 cases of oral histoplasmosis were identified from 1995-2016. Majority of them were male (89.7%). The age ranges from 29 to 85 years with mean age of 57.8 years. Almost half of them were Malays (51.3%), followed by Chinese (33.3%), Indians (7.7%), and other races (7.7%). The most common sites of oral histoplasmosis were tongue, gingiva, palate, and alveolar ridge. The main clinical presentation was ulcer (61.5%) whereas 38.5% presented clinically as swelling. 17.9% of patients were seropositive for human immunodeficiency virus (HIV), 12.8% had tuberculosis, 10.3% had diabetes mellitus, and 2.6% with hepatitis C. The incidence of oral histoplasmosis should raise suspicion of hidden immunodepression and may be the first manifestation of acquired immunodeficiency syndrome (AIDS). Early recognition and diagnosis is crucial to reduce risk of morbidity and mortality.