We present a case of NSCLC harboring ALK mutation with primary cutaneous marginal zone B-cell lymphoma (PCMZL) treated with adjuvant chemotherapy with pemetrexed and cisplatin, and ALK-echinoderm microtubule-associated protein-like 4 (EML4)-targeting treatment alectinib. Here, EML4 is linked to primary cutaneous marginal zone B-cell lymphoma.