Necrobiosis lipoidica (NL) is a rare, chronic granulomatous condition occurring mostly on the skin of the pretibial area in middle-aged woman. Only 0.3% of diabetic patients will develop NL, but about 60% of patients with NL are diabetic. NL can coexist with other diseases such as: thyroiditis, inflammatory bowel disease, sarcoidosis, obesity, dyslipidemia, hypertension, coronary heart disease, stroke, and reduced renal function. There are multiple theories regarding the etiopathogenesis of NL, but it still remains unknown. Scientific data regarding treatment for NL consists mostly of case reports and rather small clinical trials. To this day, no uniform therapeutic recommendations have been created. Topical and intralesional corticosteroids have been the first-line treatment for active, enlarging NL lesions for years. Therapies such as calcineurin inhibitors, retinoids, systemic corticosteroids, and many more have been used with various results. Currently, no single therapy method is superior to another, though some of them seem to stand out. JAK inhibitors, TNF-α inhibitors and light therapies, among others, have been showing the most promising results in NL treatment. Despite good treatment results, further studies are required to establish real efficacy and the safety of each method and create uniform therapeutic guidelines for NL treatment.