Merkel cell carcinoma (MCC) is an aggressive neuroendocrine carcinoma with a high rate of metastasis. Most MCCs present as rapidly growing red or violaceous firm nodules on the sun-exposed skin of fair-skinned Caucasians older than 60 years. MCC is unsuspected by clinical examination in 99% of cases; therefore, biopsy is necessary to establish a pathologic diagnosis. The author has experienced a 54-year-old female presenting with a 0.3cm mass on the right ear lobule. An excisional biopsy was performed, which suggested neuroendocrine carcinoma, such as Merkel cell carcinoma. During the tumor work-up, temporal bone computed tomography scan (TBCT) and magnetic resonance imaging (MRI) newly revealed a 7x5mm enhancing lymph node (LN) in right parotid gland. The LN was not clinically palpable and did not appear to be a metastatic lesion. However, the positron emission tomography-computed tomography (PET-CT) and Chest-CT revealed a solid lesion in the left upper lung(LUL) which was diagnosed with r/o adenocarcinoma (cT2aN0M0). After tumor board with otorhinolaryngology, thoracic surgery and radiology, these lesions were considered double primary cancer. In this case, the author reports MCC in earlobe with double primary cancer of adenocarcinoma in lung which went through co-operation: wide excision of the MCC in earlobe, partial parotidectomy with selective neck dissection (II-III) by the otorhinolaryngology team, and Video-assisted Thoracic Surgery of LUL by the thoracic surgery team. Pathology results showed MCC in earlobe and metastatic LN in parotid gland, whereas the lung lesion showed invasive adenocarcinoma. The patient is currently undergoing chemotherapy for the lung adenocarcinoma and followed up regularly.