Hermansky-Pudlak syndrome (HPS) is an inherited disorder that affects melanin production, blood clotting, and functions of some organs. Affected individuals typically have little or no pigment in their eyes, skins, or hair. They have poor vision, eye abnormalities, and bleeding problems. The severity of bleeding problems varies and the course is unpredictable. Some individuals have problems with the lung, intestine, kidney or heart. Progressive lung disease usually starts in the early thirties and can be fatal. Intellect is not affected by HPS. There is no cure for HPS; however medical surveillance and care may help to improve some symptoms and overall condition of life. HPS is caused by pathogenic variants in the HPS3 gene.