We present the current standard in diagnosis and treatment of basal cell carcinoma. Useful procedures for clinical management should be derived from this. A systematic literature search was carried out in the PubMed online database. The collected information was analyzed and evaluated. An overall concept was created from the gained knowledge. Basal cell carcinoma is the most common tumor in humans and its incidence is expected to increase in the future. When managing the disease, a one-dimensional orientation towards the clinical or histological subtype is not sufficient because of the heterogeneity of the tumor. The primary implementation of risk stratification, which is decisive for the further diagnostic and therapeutic steps, is becoming increasingly important. The gold standard in treatment continues to be the surgical procedure, which should be carried out using micrographically controlled surgery if possible. In addition, there are other therapeutic methods such as radiotherapy or a number of topical therapy options (photodynamic therapy, cryotherapy, application of 5-fluorouracil or imiquimod), which can be used in certain cases. Hedgehog inhibitors are also effective drugs for advanced or metastatic basal cell carcinoma. Practitioners have gained several years of experience with regard to effectiveness and handling of adverse events. With the PD-1 inhibitor cemiplimab, another therapeutic option for inoperable or metastatic tumors has been available since June 2021. Basal cell carcinoma will continue to gain in relevance in daily dermatological practice in the coming years. A structured approach to the assessment of the existing risk category of the tumor and the subsequent determination of the optimal therapy regimen are of central importance. Advanced or metastatic tumors no longer represent a hopeless situation for the patient. With long-termhedgehog therapy, an adapted dosage scheme can avoid discontinuation of therapy due to side effects. The therapeutic potential of the PD-1 inhibitor cemiplimab can also be used with the side effect profile known from other types of skin cancer.