Cutaneous intestinal either metaplasia or ectopia has mostly been described around intestinal stomas or in patients with Crohn disease. In this study, we described a unique case of cutaneous metaplasia on the elbow of a 25-year-old man who had a clinical history of epidermolysis bullosa simplex, but had no history of any gastrointestinal disease. The lesion had been clinically stable for 5 years. Histopathologically, the epithelium showed many columnar cells with mucinous cytoplasm, with no atypia or mitoses. The proliferation rate was low (7% by Ki67). The metaplastic areas expressed epithelial membrane antigen, carcinoembryonic antigen, cytokeratin 7, MUC5AC, MUC2, and Cyclin-D1.