Tehran University of Medical Sciences Basic & Clinical Cancer Research 2228-6527 14 1 2023 02 06 23 27 Behnaz Darvishi Students Research Committee, Kermanshah University of Medical Sciences, Kermanshah, Iran Negin Farhad Students Research Committee, Kermanshah University of Medical Sciences, Kermanshah, Iran Mazaher Ramezani Department of Molecular Pathology, Kermanshah University of Medical Sciences, Kermansha, Iran 2022 03 04 2022 11 29 Tuberous sclerosis complex (TSC) is a rare genetic disease that is inherited autosomal dominantly and may be associated with subependymal giant cell astrocytoma (SEGA) in 10-20% of cases. Different phenotypes are related to the form of lesions in different parts of the body, including skin, brain, kidneys, lungs, and heart. Age of patient, location of the tumor, and associated skin or neurological lesions may guide the pathologist for a definite diagnosis. Here we report a case of SEGA in an adolescent with TSC. Neurological clues including seizure and mental retardation, facial angiofibroma, renal mass, and histopathology examination of brain tumor culminated in the diagnosis of TSC and SEGA. https://bccr.tums.ac.ir/index.php/bccrj/article/view/419