Introduction: systemic lupus erythematosus is a systemic, inflammatory disease of autoimmune origin that has a wide range of clinical manifestations. It occurs more frequently in female patients and in the early age of life. Turner syndrome is a rare disease that occurs in one in every 2500-5000 live births, is clinically characterized by the presence of low size, hormonal dysfunction and multisystemic compromise; Mainly cardiac and renal involvement. Karyotype determinations along with other tests make diagnosis possible. Objective: to show the association between Turner's syndrome and systemic lupus erythematosus. Case report: the case of a 17-year-old female patient with a diagnosis of Turner's syndrome, which begins with clinical and immunological manifestations that leads to the diagnosis of systemic lupus erythematosus. Conclusions: Despite being diseases that have a genetic component in their appearance, occurring in the female sex and in the early ages of life, no relationship was found between the etiopathogenic mechanisms of both diseases.