Desmoid tumors are benign neoplasms that show locally aggressive growth but lack the potential to metastasize. Histopathologically these tumors lie between exuberant fibroproliferations and low grade fibrosarcomas and are classified as abdominal, extra-abdominal and intra-abdominal. Little is reported about clinical features and outcome of desmoids tumors of head and neck. They are rarely fatal but have a high propensity for local recurrence causing significant patient morbidity and complex patient management issues. We describe a rare case of external nasal desmoid tumor in a 10 year old male child which was managed with surgical resection and the child has been in follow up for 1 year without recurrence.