Keratosis follicularis spinulosa decalvans (KFSD) is a rare condition characterized by diffuse keratosis pilaris with a scarring alopecia of the scalp and associated photophobia, facial erythema, and palmoplantar keratoderma. Although initially described as a sex-linked disorder, several different inheritance patterns have been observed. We describe a patient whose father and sister were also affected with this condition, consistent with an autosomal dominant genetic transmission. Multiple topical and systemic treatments have been unsuccessful in this patient, attesting to the treatment refractoriness typically seen in KFSD.