Abstract: Prominent right hemidiaphragma elevation was observed on chest radiograph of a 14 years` old female patient with sickle cell disease. Her medical history yielded neither trauma nor intra-thoracic surgery. She didn`t have either motor deficit or sensation disorder on any region of her body. Thorax CT yielded no lession except the significantly elevated right diaphragma. Her cranial CT showed no lesion, too. Diagnosis of right hemidiaphragm paralysis was confirmed by positive Hitzenberg sniff test on fluoroscopy. Although several pathopysiologic mechanisms are known to be involved and lead to central neurologic complications in sickle cell disease, involvement of peripheric nerves have not been reported. Here we present a 14 years` old female patient with sickle cell anemia and unilateral diaphragm paralysis, co-existince of which have not been reported so far.