Tufted angiomas are rare lesions described as slowly growing/spreading erythematous macules especially located in the upper trunk and neck. Herein we report the case of perianal location of a tufted angioma in a young pregnant woman. She came to our observation complaining of perianal pain accompanied by bleeding at defecation. A lesion resembling a perianal fissure was observed. Mild hypertonia of the internal sphincter was confirmed at manometry. After one week of ineffective medical treatment, surgery was planed at the end of the sixteenth week under local anaesthesia. The lesion was excised and a minimal sphincterotomy was performed; histopathology report described features of a tufted angioma. The pregnancy proceeded regularly, without anal symptoms, followed by normal vaginal delivery at the thirty-eighth week. This case showed three peculiar features: the association of tufted angioma and pregnancy, the perianal location, and the clinical appearance suggestive of an anal fissure. The clinical manifestation of a perianal tufted angioma, mimicking an anal fissure, is of utmost importance to the differential diagnosis and treatment plan, especially in a pregnant woman.