Aims: Pulmonary enteric adenocarcinoma (PEAC) is a rare, aggressive variant of lung adenocarcinoma with early metastatic potential. This report presents the first case of PEAC, presenting as a solidary skull mass, and an associated literature review of the clinical presentation, histological and immunohistochemical features, and management of PEAC with skull metastasis. Methodology: We present the case of a male smoker in his 50s who presented with right-sided numbness, pain and headache. Imaging revealed a destructive skull base mass invading the right sphenoid sinus. Histopathology was consistent with PEAC. Following a multidisciplinary oncology meeting, surgical resection was deemed insufficient for achieving clear margins. The patient was treated with high-dose palliative radiation and chemotherapy. Results: Diagnosis requires thorough clinical, radiological and pathological assessment. Differentiation between metastatic PEAC versus primary sinonasal adenocarcinoma can be difficult, but histopathological markers are essential in determining the diagnosis and can have implications on treatment algorithms. Standard non-small cell lung cancer therapies are currently employed, including tumour excision and/or chemoradiotherapy. Prognosis directly correlates with clinical stage at presentation. Early diagnosis and multimodal therapy may improve outcomes; however, no standardised treatment exists for irresectable local recurrences and metastasised cases. The scarcity of PEAC literature necessitates additional research to elucidate the connections among clinicopathological features, mutations, therapies and the prognosis of PEAC. Conclusion: A careful and comprehensive evaluation is essential in the workup of skull base lesions, even in patients without known primary malignancy. This is especially important as PEAC displays early metastatic behaviour; therefore, early diagnosis and timely initiation of treatment can influence overall prognosis.