Sex cord-stromal tumors (SCST) are rare tumors of the ovary that include three main subtypes known as pure stromal tumors, pure sex cord tumors, and mixed sex-cord stromal tumors. This case involves a 25-year-old female who presented to the Gynecology outpatient clinic with a two-year history of amenorrhea associated with excessive hair growth since menarche. Ultrasonography revealed a solid left ovarian mass. Histopathology confirmed a diagnosis of SCST not otherwise specified with no cytological atypia. In this case report, we describe the process of managing this patient and a brief literature review on the updates regarding the clinical presentation, molecular changes, and management of SCSTs not otherwise specified.