Background and aim: Dermoid cysts (DC) of the head and neck are rare congenital anomalies derived from entrapment of ectodermal cells at lines of fusion in the embryo into mesoderm. Methods: We describe a 22-years-old female with an unusual presentation of DC in the subcutaneous tissue of the retro-auricular region. Results: The pathological examination of the surgically removed specimen confirmed the diagnosis of Dermoid Cysts. Conclusions: A DC in this region is rare and may be misdiagnosed as a retro-auricular lymph node. Complete excision of the lesion must be achieved with pathology study to confirm diagnosis. (www.actabiomedica.it)