Steatocystoma multiplex (MS) is a rare pathology characterized by formation of multiple sebaceous cysts in different skin areas. The article presents an overview of the pathogenesis, clinical manifestations, and course of this pathological condition. It is believed that MS is caused by mutations in KRT17 gene that encodes keratin-17. Both males and females suffer from MS. Familial cases with autosomal dominant inheritance are often observed in several generations. The disease typically manifests during adolescence, but can be detected at birth or shortly thereafter. The authors present their own clinical observation of MS in a 6-year-old child, paying attention to the issues of treatment modality and management of these patients in connection with formation of a stable cosmetic defect, reducing the quality of life of patients.