Hereditary Fibrosing Poikiloderma or POIKTMP [1,65,103] is a rare autosomal dominant multisystem disorder, characterized by skin abnormalities, the hypotrichosis of hair, eyebrows, and eyelashes, tendon contractures, myopathy, lung fibrosis, and liver disease, caused by monoallelic missense variants (with a dominant negative/gain-of-function effect) of the FAM111B gene. Here, FAM111B is linked to hereditary sclerosing poikiloderma with tendon and pulmonary involvement.