Congenital absence of the cruciate ligament is an extremely rare condition that was first reported in Giorgi's radiographic study in 1956. The authors report on a case of anterior cruciate ligament reconstruction performed on a 21-year-old female patient with congenital anterior cruciate ligament absence. We also discuss radiographic evidence that could provide clues to the congenital absence and possible difficulties that may be encountered during surgery with a review of the relevant literature.