DISCUSSION: SFTPC gene mutation is inherited in autosomal dominant pattern, but de novo mutation may occur. Various pattern of ILD has been recognized in SFTPC mutation including NSIP, usual interstitial pneumonia(UIP), and desquamative interstitial pneumonia(DIP). PNEC hyperplasia has been described to occur in association with lung inflammation, but not previously described with SFTPC mutation. We described mother and son with the same genetic mutation and similar histology of NSIP, PNEC hyperplasia and carcinoid tumorlets. Although the mutation occurred in an intron, non-transcribed portion of the gene, previous report of intron mutation led to alteration of splicing site causing abnormal protein production. Although causality between mutation and ILD cannot be firmly established, the familial nature of the disease suggested a genetic and histopathological correlation.