Hair shaft disorders encompass a wide range of hair shaft defects with or without fragility, which could be acquired or inherited genetically. Hair shaft defects may be localized or generalized and are characterized by changes in color, structure, length, and density. Pili torti, also called twisted hair, is a clinical entity in which the hair shaft is flattened and twisted by 180° along its axis. Pili annulati is described as striped hair, showing alternate light and dark bands. Trichorrhexis nodosa is the fracture of the hair shaft. Pili annulati and trichorrhexis nodosa has been reported in a single patient. Herein, we report a familial case of pili torti, pili annulati, and trichorrhexis nodosa with autosomal dominant inheritance.