Carcinoid tumors are defined as rare slow-growing neuroendocrine tumors. A majority of primary ovarian carcinoids occur in association with mature cystic teratoma or are metastatic to the ovary. A 48-year-old post-menopausal woman presented with progressive facial puffiness, and intractable diarrhea. Radiological imaging suggested a 10 × 9 × 9.2 cm right ovarian mass. She underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histopathological examination revealed primary pure carcinoid tumor of the ovary with the synchronous presence of early invasive squamous cell carcinoma of the cervix, along with the icthyosis uteri. The patient is doing well on 5 years of follow-up post-surgery without any recurrence or metastasis. In this report, we intend to highlight the rare association of cervical carcinoma and ichthyosis uteri with this tumor. In addition, we present a short review of the literature, over a decade of ovarian carcinoids associated with carcinoid heart disease.