Melanin biosynthesis is reduced in oculocutaneous albinism, an autosomal 
recessive disorder. Hermansky-Pudlak syndrome is associated with oculocutaneous 
albinism but also has systemic complications. The ocular and systemic phenotypes 
vary, depending, in part, on the genetic mutations. This report presents a case 
of a patient with Hermansky-Pudlak syndrome and the unique association of iris 
heterochromia.