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Background: Paraganglioma is a tumor derived from extra-adrenal chromaffin cells of the sympathetic paravertebral ganglia of thorax, abdomen, and pelvis. Malignancy is defined as the presence of metastases in nonchromaffin tissue (1). We discuss a case of metastatic paraganglioma for unusual course of disease and multiple challenges that arose in the management. Case presentation: 51-year-old female with mosaic turner presented to emergency with nausea, vomiting and abdominal pain. She had recent history of episodic high blood pressure and newly diagnosed diabetes mellitus. An abdominal CT scan which was done to assess abdominal pain revealed a retroperitoneal mass suspicious for paraganglioma. Subsequent blood work showed high plasma normetanephrine (x7 upper limit of normal). She underwent surgical resection of intra abdominal mass after blood pressure control with adequate alfa blockade treatment. Surgical pathology result confirmed the diagnosis of paraganglioma and showed high Ki-67 level and mitoses count. Three months post surgery, she developed severe blood pressure difficult to control; and her blood work revealed high plasma and urine normetanephrine suggestive of paraganglioma recurrence. The pre-op imaging including CT chest and abdomen were negative for any evidence of malignancy. However, repeat imaging three months post surgery showed multiple bone lesion in the skull, sternum, spine, and pelvic suggestive of metastatic disease. One week after the diagnosis of metastatic disease, she developed acute onset right eye proptosis due to retro-orbital mass which was resected. Surgical pathology was positive for metastatic paraganglioma. To determine extent of the disease and therapeutic choice, an octreotide scan was done which showed mild octreotide uptake in bone lesions. Therefore, peptide receptor radionucleotide radiation treatment (PRRT) was not considered for her metastatic disease treatment. In consultation with medical oncology, systemic chemotherapy was suggested, but it was also declined given her poor functional status. Eventually, she was started on targeted molecular therapy with sunitinib with good tolerance and response. We report this patient, first for the unusual course of disease given progression of metastatic disease over three months after surgical resection; second for the challenging therapeutic decision given the patient and disease unique characteristics. Conclusion: This case demonstrates an aggressive course of metastatic paraganglioma disease over a short period of time following surgical resection of primary tumor mass which shows the importance of earlier follow up post surgical resection of paraganglioma. This case might also justify broader work up such as MIBG or octreotide scan to determine the extent of disease and plan for the best therapeutic approach in certain cases prior to surgery.