Objective:

To report a rare case of concurrent lobular endocervical glandular hyperplasia (LEGH) and sex-cord tumor with annular tubules (SCTATs) in a patient with Peutz–Jeghers syndrome (PJS), emphasizing the rarity of this occurrence.

Key Findings:

• The patient exhibited mucocutaneous pigmentation and a history of gastrointestinal hamartomas consistent with PJS.
• Histopathological examination revealed LEGH in the cervix and SCTATs in the left ovary, with implications for malignancy risk.
• LEGH is recognized as a potential precursor lesion for malignancy in PJS patients, necessitating careful monitoring.

Interpretation:

This case highlights the diverse gynecological pathology associated with PJS and underscores the critical importance of regular surveillance for early detection of potential malignancies.

Limitations:

• The case is based on a single patient, limiting generalizability.
• Long-term follow-up data on the patient's outcomes post-surgery are not provided, which may affect the conclusions drawn.

Conclusion:

The co-occurrence of LEGH and SCTATs in this PJS patient underscores the need for vigilant gynecological monitoring in affected individuals.