The article describes the clinical case of plaque-like necrobiosis lipoidica (NL). Clinical presentation, histology, and therapy of this dermatosis are reported. According to the literature, this dermatosis may result from not only vascular lesions, which cause collagen necrobiosis at skin lesions in patients with diabetes mellitus (DM), but also various neuroendocrine, immunological, and other dysfunctions. Possible mechanisms of the development of skin lesions in DM patients include diabetic microangiopathy, hypercoagulation, lipoid collagen degeneration, inflammation, the ratio of immune complexes, trauma, and genetic predisposition. There are four clinical forms of LN: classical, scleroderma-like, granuloma annulare-like, and plague-like. We provide literature data demonstrating the need to correct carbohydrate metabolism during the treatment of NL, as well as the effectiveness of intralesional administration (by injection or phonophoresis) of corticosteroids and heparin. Patients may be advised to apply 25—30% Dimexidum solution, which enhances the permeability of biological membranes to drug substances. Further, occlusive dressings with fluorine-containing corticosteroid ointments (elocom, advantan, lorinden) are indicated. In the case of lesion ulceration, patients are sometimes subjected to the surgery (lesion resection with subsequent skin grafting). In the case of uncomplicated NL, ointments with nonsteroidal anti-inflammatory agents (indovasin, dolgit, ibuprofen) can be administered. In conclusion, it was noted that pathogenesis of NL is still an open issue and there is no systematic approach to its diagnosis and treatment. The process leads to significant cosmetic defects and reduces the quality of life of patients. The problem of an integrated algorithm of examination and treatment should be solved on both interdisciplinary (dermatology, endocrinology) and multidisciplinary level. The article is illustrated with our own photographic images of all four NL forms.