Teratomas can occur in almost any region of the body and are the most common extragonadal germ cell childhood tumors. However, craniofacial teratomas are rare. Craniofacial teratomas can present unique features and cause significant functional and aesthetic concerns. There are complex lesions that can have components intra-cranially and extra-cranially. Therefore, their management requires significant multi-stage multidisciplinary surgical procedures. Herein, we present a case of craniofacial teratoma in a child with the phenotype of proboscis lateralis that highlights some of the pertinent point of the diagnosis and management of congenital neonatal teratomas.

A 12-year-old girl with right cerebellar pilocytic astrocytoma and left Ventricular – Peritoneal (VP) shunt presented with prolonged progressive painful abdominal distention, constipation, significant weight loss and intermittent fever. Laboratory investigations including blood and CerebroSpinal Fluid (CSF) tests revealed a VP shunt infection with Brucella abortus + Brucella melitensis. Abdominal Computed Tomography (CT) scans revealed a large abdominopelvic cyst with enhancing wall suspicious for infected pseudocyst. Ovarian tumor markers including AFP, CA-125, CA 19-9 and CEA were negative. Brucella was detected in cyst fluid by Real Time -Polymerase Chain Reaction (RT -PCR). The patient had her infected shunt removed, with establishment of External Ventricular Drainage (EVD) and treatment with antibiotics. A final diagnosis of neurobrucellosis was made and she responded well to 6 months therapy of oral doxycycline, oral rifampicin and oral cotrimoxazole. This case highlights a rare occurrence of neurobrucella in patients with VP shunts and excellent response to source control and antimicrobial therapy.