A 13-year-old male presented with recurrent left nasolacrimal duct obstruction following endoscopic dacryocystorhinostomy 4 years prior at an outside institution. The past medical history was significant for stage IV neuroblastoma, diagnosed at age 2, requiring surgical resection, induction chemotherapy, autologous bone marrow transplantation and radiation, currently in remission. Preoperative CT scan demonstrated a 2 cm ovoid mass centered in the left lacrimal fossa, consistent with dacryocystocele; however, a solid tumor could not be ruled out. Subsequent surgical exploration of the lacrimal sac revealed a friable, solid mass filling the lacrimal sac, and extending into the duct. The mass was grossly resected with preservation of the lacrimal drainage system and placement of indwelling silicone stents. Histopathology confirmed the diagnosis of smooth muscle tumor of uncertain malignant potential. The patient remained free of epiphora and showed no clinical or radiographic evidence of recurrence at 6 months of follow up.