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A 23-year-old male presented with a three-week-history of crampy abdominal pain and melaena. Colonoscopy revealed a friable mass filling the entire lumen of the cecum; histologically, it was classified as perivascular epithelioid cell tumor (PEComa). An magnetic resonance imaging scan showed, in addition to the primary tumor, two large mesenteric lymph node metastases and four metastatic lesions in the liver. The patient underwent right hemicolectomy and left hemihepatectomy combined with wedge resections of metastases in the right lobe of the liver, the resection status was R0. Subsequently, the patient was treated with sirolimus. After 4 mo of adjuvant mammalian target of rapamycin inhibition he developed two new liver metastases and a local pelvic recurrence. The visible tumor formations were again excised surgically, this time the resection status was R2 with regard to the pelvic recurrence. The patient was treated with 12 cycles of doxorubicin and ifosfamide under which the disease was stable for 9 mo. The clinical course was then determined by rapid tumor growth in the pelvic cavity. Second line chemotherapy with gemcitabine and docetaxel was ineffective, and the patient died 23 mo after the onset of disease. This case report adds evidence that, in malignant PEComa, the mainstay of treatment is curative surgery. If not achievable, the effects of adjuvant or palliative chemotherapy are unpredictable.