BACKGROUND: Metastatic basal cell carcinoma (MBCC) is defined as primary cutaneous basal cell carcinoma (BCC) that spreads to distant sites as histologically similar metastatic deposits of BCC. BCCs are semimalignant, destructive, and invasive. Metastases are very rare, with an incidence of 0.0028%-0.5%. CASE REPORT: A female patient with Gorlin-Goltz syndrome and a rare case of a basal cell carcinoma with an additional regional lymph node metastasis is presented. CONCLUSIONS: This case highlights the importance of a multidisciplinary approach to and frequent monitoring of patients with Gorlin-Goltz syndrome. Early diagnosis and surgical treatment are still the treatment of first choice.