Objective: Aim of the study was to analyze 10 managed cases of Primary Histoplasmosis of Larynx, over a period of 10 years. Design: Retrospective, non-randomized, interventional case series. Patients and Method: This study was conducted in the Department of Otorhinolaryngology S.M.S Medical College from 2002 to 2011. The study consisted of evaluation of self-reported and referred patients who presented to ENT OPD and emergency. In all the cases a detailed history and clinical examination was done. All the patients underwent fiber optic laryngoscopy and subsequent direct laryngoscopy and biopsy. CT Scans were obtained for few cases. The study was a retrospective analysis of 10 treated cases. Main Outcome and Measures: Success of the treatment was defined as resolution of symptoms, differ-entiation from malignancy, no recurrence and regular follow up. Result: A total of 10 cases were managed between 2002 and 2011 and were reviewed. The main presentation was hoarseness of voice, with lesion simulating malignancy. Most common site of involvement was false cord and aryepiglottic fold (6 cases). Primary laryngeal Histoplasmosis was essentially a disease of adult males in all 10 cases. Treatment was exclusively medical with use of Itraconazole as drug of choice in 7 cases. Conclusion: Isolated laryngeal Histoplasmosis is a rare entity. Because of its simulation with malignancy it needs to be included in the differential diagnosis of neoplasm both benign and malignant. Our study clearly limits this kind of disease to adult males most commonly involving false cord and aryepiglottic fold in an endophytic pattern. Though it looks like cancer; biopsy confirms the diagnosis. The treatment is mainly medical with Itraconazole being the drug of choice.