Background: Cutaneous leiomyoma is a rare, benign smooth muscle neoplasm of the skin. According to the origin of the tumor, there are three types of cutaneous leiomyomas: piloleiomyoma, angioleiomyoma, and genital leiomyoma. We present an uncommon case of cutaneous leiomyoma which, due to clinical similarities, had been previously misdiagnosed and managed as keloid. Case description: A 26-year-old woman with Fitzpatrick skin type 4/5 visited the outpatient department of dermatology for the treatment of a painful skin lesion on the left shoulder. The symptoms were not improving from previous treatment modalities, where she was diagnosed as having a keloid. After examination we sent for a skin biopsy. The specimen was sent for histopathological and immunohistochemical investigations, which revealed cutaneous leiomyoma. Conclusion: Cutaneous leiomyoma may be misdiagnosed as a keloid. The former is of clinical relevance as they may denote hereditary leiomyomatosis and renal cell cancer. Early recognition is of paramount importance and may be life-saving.