Hamartomas are tumor-like lesions composed of tissue elements normally found at the site where they are located, but which grow in a disorganized manner. They generally occur at birth or soon after, although presentations during adult life have been reported. Hamartomas of the head and neck are very rare. The exact mechanisms behind the development of hamartomas remain unknown; however, their benign nature renders complete surgical excision sufficient for their management. Here, we describe a case of a premature infant with a hamartomatous polypoid lesion containing both fibrous and adipose components, originating from the dorsum of the nose. The differential diagnosis and management of hamartomas of the head and neck are also discussed. Although several cases series on precalcaneal fibrolipomatous hamartomas have been encountered in the literature, to the best of our knowledge, this is the first report of a nasally located congenital fibrolipomatous hamartoma.