Extensive bone marrow necrosis is a rare but severe complication of sickle cell disease. A formerly healthy man was admitted for bone pain, fever, and jaundice with severe aregenerative anemia. Bone marrow aspiration and biopsy showed extensive bone marrow necrosis while hemoglobin electrophoresis demonstrated homozygotic sickle cell disease. Despite early onset of septic arthritis of the right shoulder, outcome after blood transfusion and nonspecific treatment was favorable. Six months later, hemoglobin level remained stable up to 97 g/L. This first African case report illustrates typical features and good prognosis of extensive bone marrow necrosis in sickle cell disease. Extensive bone marrow necrosis is a highly unusual presenting manifestation of sickle cell in an adult.