Primary Ewing sarcoma of the kidney is an extremely rare yet aggressive tumour. We present a 31-year-old pregnant female who presented to the emergency department with macroscopic haematuria and an ultrasound scan demonstrating a right renal mass. On magnetic resonance imaging, the lesion had features concerning for malignancy. She underwent a radical nephrectomy and was commenced on a personally tailored chemotherapy regime designed to permit healthy delivery of her baby while adequately treating her disease. This highlights an extremely rare differential for a renal mass which should be considered in rapidly growing renal tumours of a young patient.