Tuberous sclerosis (TS) is a neurocutaneous syndrome with an autosomal dominant inheritance characterized by widespread hamartomas and benign neoplasms distributed in several organs throughout the body. The mean age of presentation is 5 years and the imaging features tend to vary with age. The most commonly affected organs include the brain, skin, retina, kidney, bones, heart and lung. The role of imaging diagnosis and management cannot be overemphasized. The aim of this paper is to highlight the specific imaging features of TS in children of African descent which is crucial to timely diagnosis and contribute to improving the outcome of the disease by avoiding common diagnostic pitfalls.