History and clinical findings: A 50-year-old woman with known tuberous sclerosis complex (TSC) was referred for further work-up of nephrotic syndrome. A left-side nephrectomy due to an anamnestically reported shrunken kidney had been performed at the age of 15 years. The patient presented with massive edematous swelling of all extremities and typical cutaneous lesions of TSC. Diagnostic findings, treatment and clinical course: Laboratory analysis revealed nephrotic proteinuria, hypalbuminemia and advanced impairment of kidney function. Computed tomography revealed a massively enlarged remaining kidney with confluent angiomyolipomata. As kidney biopsy was deemed to be too hazardous, a thorough work-up for potentially underlying diseases finally revealed endometrial carcinoma. Hysterectomy resulted in a prompt and sustained decrease of proteinuria and disappearance of edema. Conclusion: Although a renal involvement is typical in TSC the occurrence of nephrotic syndrome is not and should give reason for further evaluation.
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