Objective The aim of the study was to analyze the clinical features of patients with perianal Paget's disease (PPD) and investigate prognosis risk factors.Methods The SEER*Stat software was used to identify 116 PPD patients from 1975 to 2015 in the SEER research database. The Kaplan-Meier method was used to conduct a univariate analysis for PPD patients. The differences in survival rates were evaluated using the log-rank test. The differences in the clinicopathological features of PPD patients with or without anorectal carcinoma were compared using the chi-square test. Results The median survival time of PPD patients was 44 months. The median age of onset was 73 years old. The 43.10％ of the patients were alive at the end of follow up, and only 12.93％ of the patients died of PPD. Elderly (age＞70 years;χ2 = 9.453, P = 0.002), poor differentiation (χ2 = 46.557, P = 0.000) and abdominal perineal resection (APR;χ2 = 46.557, P = 0.000) were unfavorable risk factors of prognosis. Nearly 50％ of PPD had combined with other malignancies, and over 22.41％ of those had multiple primary neoplasms (3 or more). PPDs predisposed concurrent malignancy, and 48.21％ of PPD patients with other malignancies combined with anorectal carcinoma in the study. Stage (χ2 = 10.127, P = 0.018), and surgical method (χ2 = 12.245, P = 0.007) were statistically significant in the PPD patients with or without anorectal carcinoma. The 16.07％ of patients had multiple lesions of Paget's. Conclusion Patients with PPD have a favorable survival, while the disease-specific mortality is low. Diagnosed age, differentiation, and surgical methods were the influence factors of prognosis in PPD patients. PPDs with anorectal carcinoma is of most important in further investigation.