Background: The Bourneville Pringle disease, also called tuberous sclerosis complex (TSC) is a rare genetic disorder with dominant autosomal inheritance. It is a rare neurocutaneous disorder, which can affect numerous internal organs, for example the kidney(s), the heart, and the lungs. The formation of multiple hamartomas is causing the multiorgan systemic involvement. Case presentation: A 43-year-old patient presents with tuberous sclerosis, an autosomal dominant inherited neurocutaneous disorder which variably affects different organs, among them the kidneys. During follow-up the abdominal computer tomography (CT) showed bilateral angiolipomatosis (AML) and a fat-poor mass with a different solid aspect on the left side. A fat-poor AML can be a challenge to diagnose. It is vital to exclude malignancy to decide on further management. A CT guided biopsy was performed. The results showed an atypical renal angiomyolipoma, a rare monomorph, epitheloid type. A genetic examination was performed, which showed a mutation of the TSC2 genes. Conclusions: The Bourneville Pringle disease is a rare genetic, neurocutaneous disease, which can affect multiple organs by the formation of hamartomas, including renal manifestation as AML. In case of radiological findings of a fat poor CT component AML it is essential to keep in mind the possibility of a rare monomorph epitheloid AML and if there is any doubt, it is crucial to exclude malignancy by using biopsy. A fat-poor AML can be a challenge to diagnose. It is vital to exclude malignancy, to take the size of the kidney AML in consideration, the high risk of potentially massive hemorrhage to decide on further management.