Introduction 
Hermansky-Pudlak syndrome (HPS) is hereditary multi-system disorder characterized by oculocutaneous albinism, a bleeding diathesis, platelet storage deficiency. Individuals can also present with pulmonary fibrosis, granulomatous colitis, and/or immunodeficiency. Ocular findings include nystagmus, reduced iris pigment, reduced retinal pigment, foveal hypoplasia with significant reduction in visual acuity (usually in the range of 20/50 to 20/400), and strabismus. Hair colour ranges from white to brown; skin colour ranges from white to olive and is usually at least a shade lighter than that of other family members.(3) The storage-pool defect arises from the absence of platelet dense bodies which normally contain adenosine diphosphate (ADP), adenosine triphosphate (ATP), calcium and serotonin which are necessary to trigger the secondary aggregation response of platelets(4)
The accumulation of ceroid lipofuscin, an amorphous lipid-protein complex, is associated with the mentioned findings of pulmonary fibrosis(5)(6), and granulomatous colitis(7) which can result in increased morbidity in these individuals. HPS is more prevalent in Puerto Rico, where HPS type 1 has an estimated prevalence of 1:1800(1) and most clinical research studies have been conducted there. Worldwide HPS is rare inherited autosomal recessive disorder, with an estimated prevalence of only 1:500,000 to 1:1,000,000 in non- Puerto Rican populations(2), thus making it’s presentation in a South-Asian country such an India extremely rare. In this case report, we discuss a patient with Hermansky Pudlak Type II who was followed over a decade. This case is unique because the patient developed disseminated tuberculosis twice during the course of follow up despite compliance to treatment, implying a possible T cell defect and hence merits publication.