Fetus in fetu (FIF) is a rare congenital anomaly of asymmetric monozygotic twins, where the parasitic twin develops abnormally inside the body of the host twin. In most cases, it is incorporated into the sibling’s abdomen, which frequently presents as a retroperitoneal mass. Currently, at least 200 cases have registered worldwide, being this the first case in Nicaragua. We describe a case of a male newborn, born via cesarean section, with a history of multiple congenital malformations observed via ultrasound examination. At birth, a mass is observed on its dorsum that impresses a skull, but without the presence of bones, with three limbs, two upper and one lower, with an outline located transversely on the pelvic girdle and the presence of two male genitalia with agenesis of the testicles and an accessory kidney. A preoperative diagnosis of FIF and spinal dysraphism was made by computed tomography (CT) and magnetic resonance imaging (MRI). They shared a spinal cord and had the presence of an open spinal defect type meningocele with aberrant roots. After the diagnosis and discussion, the multidisciplinary team proceeded to surgery to perform the separation of the twin (FIF). The subsequent anatomo pathological examination revealed that the fetus was anencephalic and had reliable FIF characteristics. The resection was performed followed by the closure of the 430 mL meningocele and complete separation of the spine and the parasitic twin. We present the first case of fetus in fetu in Nicaragua and patient evolution up to the present.