Pulmonary fibrosis is broad term for a group of lung diseases, idiopathic or familial, that results in fibroblasts and excess tissue in the lungs. Because it is a generally seen in multiple generations of families, it is referred to as familial pulmonary fibrosis. The genetic aspects underlying the disease is unclear; however, there are a plethora of proposed genetic mechanisms that have been studied. The most commonly proposed genetic mechanisms, involving telomere shortening and surfactant overproduction, will be discussed in this review.