The clinical features of the sickle syndromes do not appear until after the sixth month of life, at which time most of the HbF has been replaced by HbS. Thrombo-embolic complications, retinopathy and renal papillary necrosis are more frequent in HbSC disease than in other sickle cell syndromes. First presentation of HbSC disease after the second decade is considered late in this environment. A 25 year-old Nigerian female patient is hereby presented with renal papillary necrosis as first presentation of HbSC disease. The patient presented with a sudden onset of total haematuria without history suggestive of urinary tract infection, trauma, instrumentation and significant analgesic consumption. No history of usage of herbal remedies, diabetes mellitus, sore throat, abdominal pains, skin rashes or joint pains. Physical examination did not show any characteristic habitus or findings. Findings on intravenous urography suggested renal papillary necrosis.