Cancers arising from the stromal and germ cell layers of the ovary are rare, heterogeneous, difficult to study, and require specialized multidisciplinary management. We present a clinically relevant review of the literature of these malignancies, with particular emphasis on the more typical presentations. These tumors more commonly present in younger patients and have a high cure rate. They are associated with serum markers that are informative for diagnosis and surveillance. Surgery is often part of primary treatment, with staging and preservation of fertility being important priorities. Most patients with germ cell tumors require adjuvant chemotherapy with bleomycin, etoposide, and cisplatin (BEP), as well as careful surveillance. The rarity of these tumors makes basic scientific advances more challenging.