AIM To analyze the outcomes of transanal endoscopic microsurgery(TEM) in the treatment of rare rectal condition like mesenchymal tumors, condylomas, endometriosis and melanoma. METHODS We retrospectively reviewed a twenty-three years database. Fifty-two patients were enrolled in this study. The lesions were considered suitable for TEM if they were within 20 cm from the anus. All of them underwent an accurate preoperative workup consisting in clinical examination, total colonoscopy with biopsies, endoscopic ultrasonography, and pelvic computerized tomography or pelvic magnetic resonance imaging. Operative time, intraoperative complications, rate of conversion, tumor size, postoperative morbidity, mortality, the length of hospital stay, local and distant recurrence were analyzed.RESULTS Among the 1328 patients treated by TEM in our department, the 52 patients with rectal abnormalities other than adenoma or adenocarcinoma represented 4.4%. There were 30 males(57.7%) and 22 females(42.3%). Mean age was 55 years(median = 60, range = 24-78). This series included 14(26.9%) gastrointestinal stromal tumors, 21 neuroendocrine tumors(40.4%), 1 ganglioneuroma(1.9%), 2 solitary ulcers in the rectum(3.8%), 6 cases of rectal endometriosis(11.5%), 6 cases of rectal condylomatosis(11.5%) and 2 rectal melanomas(3.8%). Mean lesion diameter was 2.7 cm(median: 4, range: 0.4-8). Mean distance from the anal verge was 9.5 cm(median: 10, range: 4-15). One patient operated for rectal melanoma developed distant metastases and died two years after the operation. We experienced 2 local recurrences(3.8%) with an overall survival equal to 97.6%(95%CI: 95%-99%) at the end of follow-up and a disease free survival of 98%(95%CI: 96%-99%).CONCLUSION We could conclude that TEM is an important therapeutical option for rectal rare conditions. AIM To analyze the outcomes of transanal endoscopic microsurgery (TEM) in the treatment of rare rectal conditions like mesenchymal tumors, condylomas, endometriosis and melanoma. METHODS We retrospectively reviewed a twenty-three years database. Fifty-two patients were enrolled in this study. The lesions were considered suitable for TEM if they were within 20 cm from the anus. All of them underwent an accurate preoperative work consisting of clinical examination, total colonoscopy with biopsies, endoscopic ultrasonography, and pelvic computerized tomography or pelvic magnetic resonance imaging. Operative time , intraoperative complications, rate of conversion, tumor size, postoperative morbidity, mortality, the length of hospital stay, local and distant recurrence were analyzed.RESULTS Among the 1328 patients treated by TEM in our department, the 52 patients with rectal abnormalities other than adenoma or adenocarcinoma represented 4.4%. There were 30 males (57.7%) and 22 females (42.3%). This series included 14 (26.9%) gastrointestinal stromal tumors, 21 neuroendocrine tumors (40.4%), 1 ganglioneuroma (1.9%), 2 solitary ulcers in the rectum (3.8%), 6 cases of rectal endometriosis (11.5%), 6 cases of rectal condylomatosis (11.5%) and 2 rectal melanomas (3.8%). Mean lesion diameter was 2.7 cm (median: 4, range: 0.4-8) Mean distance from the anal verge was 9.5 cm (median: 10, range: 4-15). One patient operated for rectal melanoma developed distant metastases and died two years after the operation. We experienced 2 local recurrences (3.8%) with an Overall survival equal to 97.6% (95% CI: 95% -99%) at the end of follow-up and a disease free survival of 98% (95% CI: 96% -99%). CONCLUSION We could conclude that TEM is an important therapeutical option for rectal rare conditions.