We report a case of a 9 year old female child with congenital pseudarthrosis of the radius. She had a history of fractures of both bones of the left forearm after trivial trauma at the age of 7 years (2003) and 8 years (2004). On each occasion she was treated conservatively in a POP cast 4 weeks. She reported to us in January 2005 complaining of a gradually increasing deformity of the left forearm. Radiologically, it was an apex anterior deformity of the distal aspect of the left radius. Clinically she had multiple café au lait spots over her body. Neurological and ophthalmological examinations were normal. The fibrous tissue and the fracture ends were excised. The fracture was stabilized with a 6-hole DCP with iliac crest graft to bridge the gap along with cortico-cancellous chips. The fracture united uneventfully at 3 months post-operatively. At 17 months post-operatively there is no evidence of recurrence of pseudarthrosis with a near normal range of movements. Congenital pseudarthrosis of the radius is an extremely rare condition with only 10 cases seems to have been reported. Dual onlay bone graft, vascularised fibular graft has been the treatment options the latter being the preferred one. But the disease being extremely rare not much has been documented about the treatment options. We treated this case by excision of the sclerotic bone ends along with a cuff of periosteum and internal fixation with DCP along with iliac strut graft to restore the length. Successful union was achieved in 3 months and the patient has satisfactory follow-up at 17 months.