Background: Umbilical skin lesions are rare but diverse and are rarely diagnosed by biopsy. Objective: This study aimed to analyze the clinical and histopathological features of umbilical skin lesions that require histopathological investigation and to aid in the diagnosis of diseases in patients with umbilical skin lesions. Methods: We performed a retrospective analysis of the patients who visited Korea University Medical center for umbilical skin lesions between January 2008 and December 2019. Age, sex, clinical features, etiologies, and histopathological features were obtained from the patients’ medical records. Results: In total, 41 cases of histologically confirmed umbilical lesions were observed. There were 29 female patients (70.73%). The lesions included hypertrophic scars (11/41), epidermal cysts (7/41), verruca (6/41), seborrheic keratosis (4/41), nevus (4/41), steatocystoma (2/41), and one case each of endometriosis, hidrocystoma, neurofibroma, soft fibroma, foreign body granuloma, tick bite, and heterotopic gastric mucosa. Conclusion: The most common umbilical skin lesions were hypertrophic scars, probably because of the increasing number of laparoscopic surgeries. Compared to the known trends in other countries, fewer biopsies were performed on the umbilical lesions for the diagnosis of metastatic cancer in this study, which might have been due to the low incidence of advanced gastric cancer and the early detection of cancer through endoscopy and computed tomography scan in Korea. Clinically, changes in treatment modality and diagnostic development can alter the frequency of the previously well-recognized diseases; therefore, medical personnel should be aware of the changing incidence of related diseases. (Korean J Dermatol 2021;59(7):501∼506)