Merkel cell carcinoma (MCC) is a rare and aggressive cutaneous malignancy with uncertain histogenesis. Here, we present an unusual presentation of giant MCC of the skin with concomitant intra-abdominal metastasis to pancreas. A 53-year-old man was admitted to our clinic with a giant ulcerated mass in the gluteal region. Incisional biopsy of the mass revealed primary poorly differentiated neuroendocrine tumor of the cutaneous origin and confirmed the diagnosis of MCC. Clinically, the patient complained of abdominal discomfort and weight loss of 15 kg over the past 4 months. Intra-abdominal computerized tomographic examination demonstrated a heterogenous-appearing giant mass of the right upper quadrant invading the superior mesenteric artery and leading to obstruction of common biliary duct and a second mass (6 cm x 6 cm) located at the inferior pole of pancreas extending toward the duodenum. As the gluteal mass was prone to trauma and leading to massive hemorrhage, excision of the gluteal mass with 2 cm of tumor-free margin was performed. V-Y advancement flap based on the perforators of the inferior gluteal and profunda femoris artery was elevated to cover the resulting defect (22 cm x 17 cm). Although the patient was referred to medical oncology for further therapeutic management, he could only survive for 11 months from his initial diagnosis. We would like to emphasize that MCC has the potential to spread to unusual organs in short intervals and prompt evaluation and proper management is essential for optimal survival of these patients.