Dermatofibroma, or benign fibrous histiocytoma, is one of the most common skin tumors; it accounts for about 3% of skin biopsy samples obtained in histological laboratories. Numerous varieties of dermatofibromas are described among which keloid type is one of the rarest. Dermatofibroma is easily diagnosed clinically if it’s a classical type represented by a small skin nodule with reddish-brown surface located on the limbs, but in atypical cases the diagnosis is difficult. Further, keloid scars occurring at the site of skin injury may have an appearance resembling dermatofibroma, as well as dermatofibrosarcoma, that explains the importance of differential diagnosis of these formations. For this reason, it is important to improve various non-invasive diagnostic methods, especially dermatoscopic examinations. The authors discuss in details dermatoscopic patterns associated with dermatofibromas and the results of histopathological examination, as well as the differential diagnosis of these formations. Possible clinical options are considered: a keloid scar may be dermatofibroma of keloid type, dermatofibroma may be a keloid scar, as well as keloid scar may transform rarely to dermatofibrosarcoma. It is important to conduct histological examination of neoplasms for differential diagnosis, take complete medical history and improve dermatoscopy skills. The option of punch biopsy in taking biomaterial should be considered in case of large formation to reduce injury, that has crucial importance if the formation turns out to be a keloid scar. Accurate diagnosis is very important, because treatment methods of keloid scar and dermatofibroma are mutually exclusive: excision is recommended in dermatofibromas (if desired by the patient with aesthetic goals), while surgery is contraindicated in keloid scars. The article is illustrated with clinical examples from the authors’ personal practice.