Hydatid cysts caused by Echinococcus species mainly involve visceral organs. Rarely skeletal involvement occurs. The spine and pelvis account for half of the cases of osseous hydatidosis, making humeral hydatid cysts one of the rarest conditions. We report a case of humeral hydatidosis in a 42-year male who presented to us with pain and swelling over the left upper arm, radiological investigations suggestive of osseous cystic lesions were inconclusive and diagnosis of hydatidosis was confirmed with a biopsy subsequently. Surgical excision and curettage of bone and surrounding soft tissue were done, and the void was filled using PMMA cement followed by antihelminthic chemotherapy. No complication or recurrence has been found postoperatively at the end of 6 months of follow-up, with preservation of limb function. To keep orthopedic surgeons aware of this morbid condition, due to its low prevalence, and advanced presentation which is likely misdiagnosed, and even after the early intervention, there is a high recurrence rate which makes this one of the rare cases of humeral hydatidosis notifiable.