Extramedullary hematopoiesis can occur during normal fetal development or as a compensatory mechanism in chronic anemia when the primary sites of hematopoiesis fail. When it occurs, it is mostly seen in sites such as the liver, spleen, and lymph nodes. Extramedullary hematopoiesis is seen in patients with abnormal morphology of red blood cells like thalassemia and abnormal red blood cell shape like hereditary spherocytosis. The incidence of extramedullary hematopoiesis in sickle cell disease is very rare. We report a case of focal adrenal extramedullary hematopoiesis in a 21-year-old patient with sickle cell disease who presented with pain in the right thorax. A retroperitoneal mass was seen in the MRI of the abdomen of the patient suggestive of extramedullary hematopoiesis. Our case highlights the importance of physician awareness of this rare pathology for proper diagnosis and management.

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