Cystic dystrophy of the duodenal wall is an uncommon complication of aberrant pancreas characterized by increased duodenal wall thickness associated with intraparietal cystic lesions. We present the case of a male patient with cystic dystrophy of the duodenal wall, which posed major diagnostic problems due to the difficulty of distinguishing this entity from tumors of the head of the pancreas. Echoendoscopy was useful in establishing the definitive diagnosis, allowing puncture-evacuation of the intracystic contents with resolution of obstructive symptoms.