Focal fibrocartilaginous dysplasia (FFCD) is an uncommon, benign bone lesion that causes angular deformities of the long bones in young children. Most deformities were seen around the knee. Diagnostic criteria are based on clinical and radiological signs: unilateral angular deformity in a long bone of a young child, associated with on X-ray a typical lucent bony defect with surrounding sclerosis at the concavity of the deformity with a cortical defect. The etiology is not understood. There were 16 cases described previously involving the upper extremity: 9 cases with FFCD of the ulna, 3 of the humerus, 2 of the radius and 2 of the phalanx. We report a case of cubitus varus deformity in a young girl of 2 years and 2 months where the deformity worsened very quickly. We believe that debridement of the fibrous tether at very young age will prevent further deformity and can correct spontaneously by remaining growth.