Myxomas in the oral cavity and lips are relatively uncommon tumors that present in different types: the first type is odontogenic myxomas and the second type is soft tissue myxomas. Odontogenic myxomas (originated from the mesenchymal cells of the oral cavity such as dental papillae, periodontium, or dental follicles) are most presented in the maxilla and mandible. In comparison, soft tissue myxomas are rare in the oral cavity and lips, and only 30 cases of unknown development were reported in the literature, and only five cases involving the lip were reported. Even if it is considered very rare, nerve sheath (soft tissue) myxoma must be included in the differential diagnosis of lip lesions and excised widely to prevent local recurrence. The aim of our case study is to present a rare case of lip myxoma in a male patient 26-year-old who presented with a soft, exophytic lesion on the lower lip.