Diaphragmatic endometriosis is a rare entity, often asymptomatic, which has been described only in small series. It is almost always associated with severe pelvic involvement. The most plausible theory about this condition is based on retrograde menstruation and subsequent transportation of viable cells in peritoneal fluid from the pelvis up the right gutter to the right hemidiaphragm, thus demonstrating its asymmetric distribution on the diaphragm. Pre-operative diagnosis is poorly supported by imaging techniques. In most cases, it is an incidental finding because the lesions may hide behind the right hepatic lobe. In that case it cannot be easily demonstrated with a laparoscope from an umbilical port. Symptomatic diaphragmatic endometriosis is associated with deep lesions which can involve the entire thickness of the diaphragm. In these cases, treatment is more difficult with possible incomplete pain relief and a considerable possibility of recurrence. In this subset, abdominal surgery is recommended. Surgical treatment must be individualized on the basis of the patient's age, fertility desires, type and location of disease and symptoms. We report the surgical treatment of a patient with synchronous pericardial, pleural and diaphragmatic endometriosis associated with pelvic peritoneal and bowel involvement. A review of the literature regarding pericardial and diaphragmatic endometriosis focusing on anatomical and surgical aspects of its management is undertaken.