Histoplasmosis has been reported in Sri Lanka for decades; however, clinical recognition and diagnosis of the disease are rare. We conducted a comprehensive search of English-language medical literature from 1969 to February 2022. Our analysis found one population survey of histoplasmin skin sensitivity testing in 1969 (5.7% positive incidence) and ten cases of histoplasmosis across Sri Lanka. The highest number of cases were reported from the Central province, where the positive histoplasmin tests were observed previously. None of the patients were positive for HIV, and both diabetes and betel chewing seem linked with oral histoplasmosis. Out of the reported cases, 50% were diagnosed with disseminated histoplasmosis; asymptomatic, acute pulmonary, and chronic pulmonary histoplasmosis were not observed. The clinical presentation varied from oral lesions (the most common presentation), skin lesions, and fever of unknown origin to adrenal crisis. Fungal culture and histopathology were used for diagnosis, with no use of antigen and antibody testing. Amphotericin B and itraconazole were used as treatment options. The rising at-risk population mandates enhancing laboratory diagnostic facilities and increasing the awareness of medical professionals in Sri Lanka on histoplasmosis.