Immature teratoma of the ovary is a rare malignant germ cell tumor whose etiology is unknown. Preoperative diagnosis and treatment of this tumor can be challenging for clinicians. We present a 1-year and 9-month female child who presented with a 1- month history of progressive nontender abdominal distension. Computed tomography scan of the abdomen revealed a huge well-defined heterogenous mass arising from the peritoneal cavity. Surgical resection was performed. Histopathology coupled with immunohistochemical analysis of the specimen confirmed it to be an ovarian immature teratoma, grade one. The child recovered well postoperatively. Surgery alone is curative for most children and adolescents with resectable ovarian immature teratoma thus avoiding the long-term effects of chemotherapy in most children with this disease.
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