Background: A 31-year-old woman with tuberous sclerosis complex presented with a 1 week history of subjective fever, chills, rigors, poor appetite and dizziness.
Investigations: Physical examination, urine and blood analysis, CT of the abdomen, chest and brain, and chest X-ray.
Diagnosis: End-stage renal disease, septic shock and urinary tract infection secondary to huge bilateral angiomyolipomas of the kidney associated with tuberous sclerosis complex.
Management: Antibiotic therapy, vasopressor treatment and bilateral nephrectomy, followed by hemodialysis while awaiting renal transplantation.