Metastatic Crohn’s disease (MCD) is the rarest yet one of the most disabling cutaneous manifestations of inflammatory bowel disease. Granulomatous lesions frequently mimic infections, autoimmune dermatoses, or drug reactions, causing delayed diagnosis and substantial psychosocial burden. Its true incidence is probably underestimated, as lesions may precede, accompany, or follow intestinal Crohn’s disease and often arise in intimate locations. This review synthesises current knowledge on epidemiology, pathogenesis, and clinical–histological features, emphasising emerging mechanisms such as dysregulated Th17/IL-23/TNF-a signalling, NOD2 variants, epitope spreading, and aberrant T-cell homing. We present the 2025 Delphi diagnostic criteria for oral and genital/other MCD and concise differential-diagnosis frameworks. Treatment evidence is summarised from topical and systemic therapies to biologics, IL-23 inhibitors, and JAK blockade. Finally, we introduce DUET-MCD, a 24-month stepwise dermatology – gastroenterology management scheme designed to standardise care and reduce diagnostic delay – prompting the question of how many “refractory” dermatoses represent unrecognised MCD.