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Primary pulmonary mucoepidermoid carcinoma (MEC) is extremely rare in children and is characterized by a mucus-secreting and squamous cell combination. Solid pseudopapillary neoplasm of the pancreas is a rare tumor of the pancreas, which is mostly seen in young women. We present the case of an 8-year-old boy previously diagnosed and treated by lung lobectomy for MEC. He was admitted to our hospital with an incidentally recognized mass at the pancreas during postoperative workout studies. Distal pancreatectomy was performed. Surgery was curative for both tumors. To the best of our knowledge, this is the first reported case of pseudopapillary neoplasm concomitant with MEC in the literature.