Congenital pseudoarthrosis of the ulna and contralateral fibula occurred in a seven-year-old boy. Multiple limb involvement is extremely rare and has only been reported involving the tibia and fibula. Congenital pseudarthrosis of the tibia is a well-known entity, with other bones affected much more rarely. A case is reported involving the ulna and the contralateral fibula. This appears to be the first case of congenital pseudarthrosis reported involving an upper and a lower limb.