BACKGROUND: Perivascular epithelioid cell tumor (PEComa) of the colorectum is exceedingly rare, with only a few published reports. It presents with a wide spectrum of biological behavior, ranging from benign to malignant. The prognosis for malignant PEComa is poor and there is little consensus on its treatment. AIM: To fully characterize PEComa and standardize its diagnosis and treatment. METHODS: Patients with colorectal malignant PEComa were identified from the First Affiliated Hospital, Zhejiang University School of Medicine and People's Hospital of Anji. Cases with controversial pathology and cases lost to follow-up were excluded, leaving seven remaining cases that formed the basis of the study. We collected relevant clinicopathological, therapeutic and followup details. Disease stage and progression were assessed by contrast-enhanced computed tomography at baseline and at 3-month intervals. RESULTS: The mean age was 43 years, with a range of 5 years to 73 years. The average body mass index was 21.8 ± 3.0 kg/m CONCLUSION: Radical or extended resection is the key to prolonged survival of malignant PEComa. More meaningful studies are urgently needed to establish the standardized diagnosis and treatment.