The case is presented of a 1.5-year-old with the typical CT features of tuberous sclerosis and pachygyria, in combination. Tuberous sclerosis was evidenced by multiple subependymal calcifications, and pachygyria by the relevant CT appearances. Periventricular heterotopia and total callosal agenesis were associated findings. The findings in this patient convincingly suggest a close relationship between tuberous sclerosis and pachygyria, although the case is the first to be reported in the literature.