Ovarian yolk sac tumors (OYSTs), also known as endodermal sinus tumors, are rare and highly malignant germ cell tumors, accounting for approximately 1% of all ovarian cancers. They predominantly affect children and young adults, with a rapid growth rate and early metastasis, making early diagnosis and treatment crucial. This report presents the case of a 6‐year‐old female from a low‐resource setting who initially presented with symptoms suggestive of acute appendicitis, including abdominal pain, fever, and vomiting. This was supported by a CT scan; however, intraoperative findings revealed a large right ovarian mass, which was surgically excised via cystectomy and histologically confirmed as an OYST. The patient recovered well postoperatively and was referred for oncological management. This case underscores the importance of considering OYSTs in the differential diagnosis of pediatric abdominal masses and highlights the necessity of a multidisciplinary approach for optimal patient outcomes. Enhanced awareness and improved diagnostic strategies are essential for better management of this rare tumor, particularly in resource‐limited settings.