The clinical case of Muir-Torre syndrome in a patient with multifocal synchronous basal cell skin cancer with sebaceous differentiation and endometrial cancer in a medical history, including clinical manifestations and dermatoscopic pattern features of sebaceous adenoma, epithelioma with sebaceous glandular differentiation and sebaceous glandular skin hyperplasia, which were confirmed by pathomorphological examination, is described. Muir-Torre syndrome is a rare genetic syndrome with autosomal dominant type of inheritance, characterized by a combination of multiple sebaceous neoplasms and oncopathology of visceral organs. The disease is found in patients over 50 years old, mainly in men. The etiology of syndrome is associated with mutations in the genes of DNA repair system leading to skin and visceral organs cancers. The dermatological spectrum of Muir-Torre syndrome is presented by benign and malignant skin cancers with sebaceous differentiation, namely adenoma, sebaceoma, superficial epithelioma, sebaceous carcinoma and basal cell cancer. Patients have a malignant cancers of visceral organs, namely colon, rectum, urogenital and bronchopulmonary systems, gastrointestinal and biliary tracts, mammary gland and blood system in addition to skin lesions. In most cases, skin symptoms of disease debut after visceral organs cancer detection. The diagnostic criterion of Muir—Torre syndrome is the association of multiple sebaceous glandular skin neoplasms with cancer of visceral organs. Additional clinical signs include sebaceous glandular hyperplasia of the skin. In case of evidence detection of skin lesions, a careful examination of patient must be done for the purpose of timely detection of visceral organs’ oncopathogy and medical and genetic consultation of both the patient and his close relatives.