IntroductionPrimary retroperitoneal mucinous cystadenoma is an extremely uncommon tumor occurring mostly in females. The histogenesis of PRMC remains unclear and Open surgery is the most impressive treatment.Case presentationWe present a 20-year-old Iranian woman with history of intermittent abdominal pain. In physical examination, her abdomen had a mildly asymmetrical distention and a round shape mass was palpated in right abdomen also she had a mild tenderness in right abdomen. Radiologic assessment revealed a right retroperitoneal smooth cystic mass (20 × 15 cm) without invasive features. The patient underwent complete surgical excision of the tumor by a laparotomic approach because of its size. The lesion was gently dissected from the contiguous organs and removed completely without spillage of its content. In microscopic investigations, sections showed a unilocular cyst with fibrous wall lined via monolayered bland looking columnar mucinous epithelium and no atypia or stromal invasion was presented. Diagnosis of a PRMC was made. The patient has discharged without any complications in 2 days' postoperative course.DiscussionGenerally, cysts are asymptomatic and are found fortuitously after a routine checkup assessment. Symptoms and signs related to this cyst are mainly because of their huge size and compression effect. Radiological assessments are effective in specifying the lesion preoperatively; although, the final diagnosis always needs histopathological verification.ConclusionWhen the tumor is diagnosed, full resection should be considered because of the infectious or malignant potential of the tumor.