Sickle cell disease is the most common inherited blood disorder in the United States. The primary driver of pathology is microvascular occlusion which affects multiple organ systems including the kidney. The renal pathology usually manifests as hematuria, proteinuria, or microalbuminuria, and up to 10% of individuals with homozygous sickle cell disease (HbSS) develop renal failure over their lifetime. At ultrasound, the most common finding is increased size with mild variation in echogenicity of the renal parenchyma. We report the ultrasound appearance of a case of acute sickle cell nephropathy with markedly abnormal, enlarged, and echogenic kidneys due to intravascular hemolysis and hemosiderosis, confirmed by biopsy. Knowledge of this potential presentation of sickle cell nephropathy may help aid in earlier diagnosis of renal complications and avoidance of unnecessary renal biopsies.