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Introduction: Tuberous sclerosis (TS) is a phacomatosis characterized by the development of benign tumors of the skin, kidney, heart, eye and brain .The objective of this paper is to discuss the clinical and evolutionary aspects of this condition using 18 representative cases. Materials and methods:This is a retrospective study of 18 cases of children with TS compiled in the Neuro-Pediatrics unit of the Children's Hospital of Rabat during the period from 2004 to 2012. Results: The 18 cases included 8 boys and 10 girls and their ages ranged from 17 months to 16 years. Neurological involvement was present in 17 patients; 4 patients had West syndrome, and 13 patients had partial or generalized seizures. Mental retardation was noted in 7 patients and autism in 4. Brain imaging noted 12 cases of parenchymal calcifications, 4 cases of subependymal nodules and 2 cases of tubal cortical. Achromic or angiofibromatous lesions were found in 11 patients. Renal ultrasound was performed in 12 patients and 9 cases of parenchymal abnormalities such as angiomyolipoma and polycystic kidney disease were discovered. Cardiac involvement with intracardiac hamartoma was noted in one patient. Patient outcome was variable depending on the organs affected. Conclusion:TS is a rare phacomatosis. Renal and cardiac evaluations are needed to rule out the risk of associated life-threatening illness. Timely multidisciplinary care is necessary to mitigate severe impact of this debilitating disease.

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