Sturge-Weber Syndrome (SWS) occurs sporadically with a frequency of approximately 1 in 50,000. SWS is a mesodermal phakomatosis. Klippel-Trenaunay Weber syndrome (KTWS) is another very rare phakomatosis. Overlap between SWS & KTWS is very rarely encountered. We report a 19 months old boy with features of both SWS and KTWS. The reported case had seizures, port wine haemangioma of the right side of the body, glaucoma of both eyes, subcortical calcification which were consistent with the Sturge Weber Syndrome; on the other hand he had also hypertrophy of the right side of the including the face and limbs, angiomatous skin naevus, varicosities consistent with the KTWS.