A case is described in which self-mutilation of the genitalia by a female patient was associated with the development of schizophrenia. The psychopathology was dominated by sexual themes and resembled reports of patients with erotomania in the setting of a paranoid or schizophrenic psychosis. The literature on genital mutilation by female and male patients is reviewed briefly. It is suggested that such behaviour in women indicates severely disturbed psychosexual development but lacks diagnostic significance.