Some hotspot mutations of <i>KIT</i> have been reported to be associated with mastocytosis and GISTs, while the relationship between <i>KIT</i> mutations and hyperpigmentation and lentigines has not been fully elucidated.<h4>Methods</h4>In this study, we presented a three-generation Chinese pedigree with progressive hyperpigmentation and generalized lentigines inherited in an autosomal dominant pattern. The gene discussed is KIT; the disease is mastocytosis.