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Primary venous aneurysms are rare and usually asymptomatic. Venous aneurysms are manifested more frequently in the lower extremities than in the upper extremities. Primary venous aneurysms of the upper extremities are more often reported as aesthetically displeasing bulges or incidental findings. Here, we report the rare case of an axillary primary venous aneurysm in a pediatric patient who presented with syncope and massive pulmonary embolism and highlight the management.