Background: Ovarian metastases, which may simulate primary ovarian neoplasms, can occur in advanced stage primary cervical adenocarcinoma (AC). However, such tumors are very rarely observed in cases of endocervical adenocarcinoma in situ (AIS) with early or even no obvious signs of tumor invasion. Case: We present a case of ovarian metastasis occurring in a 37-year-old triparous premenopausal woman with a diagnosed case of endocervical AIS. The patient presented with a rapidly growing and painful abdominal mass 22 months after a cold knife cone biopsy and 19 months after a simple extrafascial hysterectomy. She was subsequently treated with a combination of surgery and chemotherapy and is currently free of disease seven years after the initial diagnosis of endocervical AIS lacking unequivocally recognizable stromal invasion. Conclusion: Endocervical AIS with early or even no obvious detectable invasion may spread to the ovaries, but this situation is exceptionally rare. We propose that, in such cases, ovary sparing surgery is justifiable for initial treatment. Medical care strategies for patients affected by rare diseases largely depend on experience gained from reported cases in published literature, this is what compelled us to report this particular case.