Background: Disseminated histoplasmosis is a rare fungal infection and most documented cases are found in immune–compromised individuals especially HIV infection.

Objective: To describe a case of progressive disseminated Histoplasmosis in an HIV reactive adolescent girl with fever and rash presenting as HLH.

Case study: We report a case of disseminated histoplasmosis in a 12 year old HIV reactive adolescent girl, admitted with PUO, skin rash, a short travel history without any known illness in the past.

Conclusion: Histoplasmosis should be considered as a differential diagnosis in HIV reactive patients with HLH irrespective of the endemicity.