ABSTRACT
Tuberous sclerosis complex (TSC) is a common neurocutaneous disorder characterized by hamartomatous changes in the lungs, brain, kidneys, skin, heart, and other organs. This retrospective study was done to see the clinical presentation and neuro imaging pattern of TSC in a tertiary care centre of Bangladesh and was conducted at Pediatric Neurology Unit of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from January 2013 to December 2013. Among total 10 patients male-female ratio was 3:2. Commonest age group was 1-5 year in 50%. Common cutaneous abnormality was facial angiofibroma in 100%. Commonest neurological presentation was epilepsy in100%, ASD in 20% patient. Common renal manifestation was multicystic kidney disease (20%). CT/MRI findings were periventricular calcifications in 70% patients. In this study facial angiofibroma is the most common skin manifestation and epilepsy is the common neurological presentationandperiventricular calcification is the most common neuro-radiological findings in tuberous sclerosis complex.