Perineal endometriosis with anal sphincter involvement is a rare occurrence with only nine cases reported so far. Two such cases are presented, and the literature is reviewed. In presented cases, diagnosis was suspected at clinical exam. Anal manometry was performed in both cases and endoanal ultrasound in one case. Wide surgical excision of endometriotic mass together with part of external anal sphincter was carried out in both cases. The procedure was followed by anal sphincter reconstruction in an "overlapping" fashion in the first and "apposition" technique in the second case. Histopathologic tests confirmed endometriosis. The recovery was uneventful in both cases with excellent functional results. Two years after the operation, patients are asymptomatic and fully continent. According to the literature and our own experience, wide excision of endometrioma with primary sphincteroplasty seems to be the best chance of cure with satisfactory functional results and should be recommended.