Fibrodysplasia ossificans progressiva (FOP) is a rare and disabling genetic disorder characterized by congenital malformation of the great toes and by progressive heterotopic ossification. There is no effective treatment. Conservative management is unsuccessful, and operation result in failure because new ectopic bone forms at the operative site. We report a 10-year-old boy with FOP who underwent surgical management combined with non-steroidal anti-inflammatory drugs (NSAIDs).