Human cases due to Salmonella enterica subsp. arizonae are especially rare, but it may affect immunocompromised patients and infants. We present a case of endocarditis in a patient with sickle cell disease and a review of earlier cases caused by this rare human pathogen. The patient was successfully treated with ceftriaxone and ciprofloxacin. There are only few cases of salmonella endocarditis reported in the last six decades and it is the first case of Salmonella enterica subsp. arizonae endocarditis in the literature to the best of our knowledge.