<b><i>Introduction:</i></b> Koenen tumors are benign, cutaneous manifestations of tuberous sclerosis. These are disfiguring, painful, and challenging to treat as they frequently recur. We report a case of long-standing, multiple Koenen tumors affecting all twenty nails in an elderly female who was successfully treated with a combination of topical sirolimus 1%, surgical excision, and electrofulguration. <b><i>Case Report:</i></b> A 57-year-old lady presented with multiple, asymptomatic periungual, and subungual tumors affecting all twenty nails since 27 years. Cutaneous examination revealed confetti macules, ash-leaf macule, and shagreen patch over trunk. Nail biopsy was compatible with Koenen’s tumor. Computerized tomography of brain showed diffuse patchy sclerosis. The tumors were treated with topical sirolimus 1% ointment for 10 months with excellent regression. Electro­fulguration for both great toenails and surgical excision of right thumbnail periungual fibroma was done. 1% sirolimus was advised after the surgical treatment. There were no adverse effects or recurrence of tumors over a 2-year follow-up. <b><i>Discussion:</i></b> Topical sirolimus 1% was effective in tumor regression and preventing new tumor formation. Larger tumors that interfered in daily chores were treated with excision and electrofulguration. Thus, a combination treatment for this rare presentation of tuberous sclerosis provided optimum results.

Rhabdomyomas are histologically benign tumors known to be associated with tuberous sclerosis. The natural history predicts the majority of tumors to be asymptomatic and regress within the first year of life. We describe a neonate presenting on day 1 of life with cardiovascular collapse secondary to a massive rhabdomyoma. Surgical resection was excluded due to the extensive nature of the lesion and oral sirolimus, a mammalian target of rapamycin inhibitor, was commenced to promote tumor regression. The patient developed intractable arrhythmias requiring extracorporeal life support during therapy.

Ameloblastic fibro-odontoma (AFO) is a benign, mixed odontogenic tumor of epithelial and mesenchymal origin. It is predominantly seen in the younger age group, with delayed or altered eruption of teeth. Being clinically asymptomatic, this is identified incidentally during routine radiographic examination. Although considered to be nonaggressive with minimal tendency for recurrence, AFO requires surgical excision with long-term follow-up. This paper presents a rare case report of an 11-year-old boy, who was provisionally diagnosed with complex odontoma and later turned out to be AFO of the mandible.