: Introduction: Malignant pheochromocytoma is a rare neuroendocrine tumor with an annual incidence of 3–8 cases per 1 million per year in the general population. Its clinical presentation can vary greatly. There are few published cases of giant pheochromocytomas. We report a case of the largest tumor in the world. The literature is reviewed to evaluate the spectrum of disease presentations and the available options for disease management and treatment. Case presentation: A 52-year-old male, originally from El Kelaa des Sraghna in the middle of Morocco, which had a 36cm mass of the left suprarenal gland with metastatic lesions. He was treated with a combination chemotherapy regimen of cyclophosphamide, vincristine and dacarbazine. Conclusion: A heterogeneous clinical presentation of malignant pheochromocytoma and a short response to chemotherapy had been observed in our case. However, controlled studies need to determine a standard of care.