Introduction: Sarcoidosis (SA) is a multisystemic, granulomatous disease with unknown etiology. Infectious and non-infectious, genetic factors, as well autoimmunity have been considered as potential etiologic causes. Pulmonary involvement is the most prominent, however other locations are not infrequent.

Clinical Case Description:  We have reported a case of a 30 years old Caucasian female, diagnosed with peritoneal sarcoidosis. The patient was asymptomatic, and was undergoing diagnostic procedures due to infertility. Transvaginal ultrasonography revealed non- characteristic changes localized proximal to the ovary and presence of a little amount of free fluid inside the peritoneal cavity. Due to subsequent high levels of serum CA-125 marker a suspicion of asymptomatic ovarian cancer was made. Results of histological examination of samples obtained from a diagnostic laparoscopy showed non-caseating granulomas in the peritoneum of the abdominal cavity, ovaries, oviducts, uterine body, and large intestine. Tuberculosis and carcinoma were excluded as a potential etiology of these findings. HR-CT scan of lungs showed no pathology but a diagnostic fiberoscopy with biopsy of the wall of the bronchi showed presence of sarcoid granulomas.

Conclusions:  This case has a potential scientific as well as clinical value, as it describes a case of peritoneal sarcoidosis, which is an extremely rare extrapulmonary manifestation of the disease, without any parenchyma lung involvement. The elevated level of CA-125 in sarcoidosis is a subject, which deserves a further study on a larger number of patients, as it has already been reported in a few cases.